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Total anomalous pulmonary venous connection

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor-In-Chief:Cafer Zorkun, M.D., Ph.D. [2]; Priyamvada Singh, MBBS [3]; Sahar Memar Montazerin, M.D.[4] Assistant Editor-In-Chief: Kristin Feeney, B.S. [5]

Synonyms and Keywords: TAPVR, Anomalous pulmonary venous connection, anomalous pulmonary venous drainage, anomalous pulmonary venous return

Overview

Overview


Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Maheep Singh Sangha, M.B.B.S. Sahar Memar Montazerin, M.D.[2]

Overview

Total anomalous pulmonary venous connection (TAPVC) is a rare cyanotic congenital heart defect (CHD) in which all four pulmonary veins are malpositioned and make anomalous connections to the systemic venous circulation. (Normally, pulmonary venous return carries oxygenated blood to the left atrium and to the rest of the body). A patent foramen ovale or an atrial septal defect must be present in order to allow systemic blood flow.

Historical Perspective

Total anomalous pulmonary venous connection was first described by Dr. Winslow, in 1739. The first description of the anatomic characteristics of total anomalous pulmonary venous connection was done by Dr. Brody in 1942. Anatomic description was further completed by another article authored by Dr. Darling in 1957. The first antemortem diagnosis was made in 1950. The first successful surgical repair was performed by Dr. Lewis, surgeon, in 1956.

Classification

Total anomalous venous connection (TAPVC) is classified into four subtypes based on the location of pulmonary venous drainage. These subtypes include, supracardiac, cardiac, infracardiac, and mixed. Supracardiac (type I) is the most common form. Pulmonary venous obstruction is usually seen in infracardiac subtype though. Smith classification is another system that classified this disorder to two categories based on the presence of pulmonary obstruction and the location of anastomosis in relation to diaphragm.

Pathophysiology

In patients of Total anomalous pulmonary venous connection (TAPVC) there is a mixing of oxygenated pulmonary venous blood with deoxygenated blood from systemic circulation.

Causes

Like many other congenital heart defects, the exact cause of total anomalous pulmonary venous connection is not known. Few factors like genetics, maternal alcohol syndrome and some drugs have been found to occur with increased frequency.

Differential Diagnosis

Total anomalous pulmonary venous connection should be differentiated from other cyanotic congenital heart diseases found in the pediatrics population. These disorders include tetralogy of Fallot, persistent truncus arteriosus, transposition of the great vessels, tricuspid atresia, trilogy of Fallot, and pentalogy of Fallot.

Epidemiology and Demographics

Total anomalous pulmonary venous connection is a rare cyanotic congenital heart disease and occurs in 6 to 12 per 100,000 live births.

Risk Factors

There are certain factors that increase the risk of congenital heart diseases, including maternal obesity, medication during pregnancy, alcohol and drugs, rubella during pregnancy, diabetes during pregnancy, and genetic factors.

Screening

There is insufficient evidence to recommend routine screening for total anomalous pulmonary venous connection.

Natural History, Complications and Prognosis

The natural history of untreated patients of TAPVC is not very favorable. It depends on the type of obstruction and amount of shunting across the atrium.

Diagnosis

History and Symptoms

The clinical features in total anomalous pulmonary venous connection depend on the type of anatomic variant present in the patient. This, in turn, determines the amount of mixing between the pulmonary and systemic circulation. In patients with obstructed TAPVC, pulmonary venous circulation drains into the systemic venous circulation. This causes increased returns to the right side of the heart and pulmonary hypertension that can manifest as cyanosis, dyspnea, pulmonary edema, respiratory failure, shock, and hypotension. In patients with unobstructed TAPVC clinical findings are quite similar to conditions with left-to-right shunting like dyspnea, difficulties in feeding, and failure to thrive.

Physical Examination

The physical findings depend on the degree of obstruction and the degree of left-to-right shunting. Physical examination of patients may be remarkable for a decreased pulse, hypotension, tachypnea, peripheral edema, S3 gallop, diastolic murmur due to tricuspid regurgitation, hepatomegaly, and cyanosis.

Laboratory Findings

There are no particular laboratory findings associated with the total anomalous pulmonary venous connection, however, hypoxia, acidosis, and hypercarbia may be observed in severe cases.

Electrocardiogram

Electrocardiography findings are not very specific. However, they can show changes due to the dilatation of the right side of the heart. Possible findings include tall P wave, right axis deviation, and ST changes corresponding to right ventricular hypertrophy.

X-ray

The findings on chest radiography vary depending on the type of anatomic variant of total anomalous pulmonary venous connection (TAPVC). Snowman sign is considered pathognomonic for the diagnosis of total anomalous pulmonary venous connection.

MRI

Magnetic resonance imaging can be helpful as a diagnostic tool in conditions where the echocardiographic findings are inconclusive.

CT

Computed tomography can be helpful as a diagnostic tool in conditions where the echocardiographic findings are inconclusive.

Echocardiography

2D echocardiography along with doppler ultrasonography is a useful tool to diagnose total anomalous pulmonary venous connection.

Other Imaging Findings

In patients with total anomalous pulmonary venous connection, cardiac catheterization can be used as a diagnostic and therapeutic modality. However, with the availability of better non-invasive tools the use of these are decreasing.

Treatment

Medical Therapy

Medical therapy are primarily used to stabilize the patient of total anomalous pulmonary venous connection.

Surgery

Surgery is the mainstay of treatment in total anomalous pulmonary venous connection and should be performed as soon as possible. The surgical procedure varies depending upon the anatomy of the TAPVC lesion.

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Classification

Classification


Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Raviteja Guddeti, M.B.B.S. [2] Sahar Memar Montazerin, M.D.[3]

Overview

Total anomalous venous connection (TAPVC) is classified into four subtypes based on the location of pulmonary venous drainage. These subtypes include, supracardiac, cardiac, infracardiac, and mixed. Supracardiac (type I) is the most common form. Pulmonary venous obstruction is usually seen in infracardiac subtype though. Smith classification is another system that classified this disorder to two categories based on the presence of pulmonary obstruction and the location of anastomosis in relation to diaphragm.

Classification

A common classification system for total anomalous venous connection (TAPVC) is as the following:[1][2][3]

Image

[5]

Another system classifies TAPVC into two types depending on the obstruction of pulmonary veins.

Type Site of drainage
Supracardiac (type I)
  • Hemiazygos vein
Cardiac (type II)
Infracardiac (type III)
  • Smith classification is another system that classified this disorder to two categories based on the presence of pulmonary obstruction and the location of anastomosis in relation to diaphragm:[6]
    • Supradiaphragmatic without [[pulmonary] venous obstruction
    • infradiaphragmatic with pulmonary venous obstruction

References=

  1. ↑ Alam, Tariq; Hamidi, Hidayatullah; Hoshang, Mer Mahmood Shah (2016). “Computed tomography features of supracardiac total anomalous pulmonary venous connection in an infant”. Radiology Case Reports. 11 (3): 134–137. doi:10.1016/j.radcr.2016.04.005. ISSN 1930-0433.
  2. ↑ Hines, Michael H.; Hammon, John W. (2001). “Anatomy of Total Anomalous Pulmonary Venous Connection”. Operative Techniques in Thoracic and Cardiovascular Surgery. 6 (1): 2–7. doi:10.1053/otct.2001.22696. ISSN 1522-2942.
  3. ↑ CRAIG JM, DARLING RC, ROTHNEY WB (1957). “Total pulmonary venous drainage into the right side of the heart; report of 17 autopsied cases not associated with other major cardiovascular anomalies”. Lab. Invest. 6 (1): 44–64. PMID 13386206.
  4. ↑ Singh, N.; Singh, R.; Aga, P.; Singh, S. K. (2013). “Cardiac type of total anomalous pulmonary venous connection: diagnosis and demonstration by multidetector CT angiography”. Case Reports. 2013 (jan03 1): bcr2012007994–bcr2012007994. doi:10.1136/bcr-2012-007994. ISSN 1757-790X.
  5. ↑ Case courtesy of Dr Vincent Tatco, Radiopaedia.org, rID: 51911
  6. ↑ Smith, Blanca (1961). “Total Anomalous Pulmonary Venous Return”. American Journal of Diseases of Children. 101 (1): 41. doi:10.1001/archpedi.1961.04020020043008. ISSN 0002-922X.


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Pathophysiology

Pathophysiology


Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Cafer Zorkun, M.D., Ph.D. [2]; Keri Shafer, M.D. [3]; Priyamvada Singh, MBBS[4]; Assistant Editor-In-Chief: Kristin Feeney, B.S. [5]

Overview

In patients of total anomalous pulmonary venous connection (TAPVC) there is a mixing of oxygenated pulmonary venous blood with deoxygenated blood from systemic circulation.

Pathophysiology

Mixing of blood could occur at three levels i.e. supracardiac, infracardiac and cardiac. In the former two the mixing occurs outside the heart and in latter inside the heart (right atrium).

This mixed deoxygenated blood is shunted from right-to-left side of heart (patent foramen ovale, atrial septal defect or patent ductus arteriosus). This right to left shunting causes cyanosis in the patient.

Since, the right side of heart is receiving blood both from pulmonary and systemic circulation it leads to development of pulmonary hypertension, right atrial and ventricular hypertrophy.

Obstructive forms: In the supracardiac form of TAPVC, the obstruction can occur by compression of the ascending vertical vein between the left main stem bronchus and left pulmonary artery. It can also occur as narrowing at the insertion of the vertical vein into the innominate vein. Obstruction is present in almost all cases of infracardiac TAPVC but is uncommon in the cardiac form.

Images

Normal heart[1]
Total anomalous pulmonary venous connection[2]

Genetic

Genetic mutations associated with total anomalous pulmonary venous connection include:[3][4]

Associated Disorders

Gross Pathology

cardiac type of TAPVC with opening of cardiac vein into right atrium
supracardiac type of TAPVC with connecting vertical vein



Video

Unobstructed forms: No significant stenosis of the pulmonary veins. {{#ev:youtube|eODH6E_OA_M}}

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References

  1. ↑ https://www.cdc.gov/ncbddd/heartdefects/tapvr.html
  2. ↑ https://commons.wikimedia.org/wiki/File:Tapv-575px.jpg#/media/File:Tapv-575px.jpg
  3. ↑ Phelan, K.; McDermid, H.E. (2011). “The 22q13.3 Deletion Syndrome (Phelan-McDermid Syndrome)”. Molecular Syndromology. doi:10.1159/000334260. ISSN 1661-8777.
  4. ↑ Bleyl, Steven B.; Saijoh, Yukio; Bax, Noortje A.M.; Gittenberger-de Groot, Adriana C.; Wisse, Lambertus J.; Chapman, Susan C.; Hunter, Jennifer; Shiratori, Hidetaka; Hamada, Hiroshi; Yamada, Shigehito; Shiota, Kohei; Klewer, Scott E.; Leppert, Mark F.; Schoenwolf, Gary C. (2010). “Dysregulation of the PDGFRA gene causes inflow tract anomalies including TAPVR: integrating evidence from human genetics and model organisms”. Human Molecular Genetics. 19 (7): 1286–1301. doi:10.1093/hmg/ddq005. ISSN 0964-6906.

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Causes

Causes


Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Cafer Zorkun, M.D., Ph.D. [2]; Keri Shafer, M.D. [3]; Priyamvada Singh, MBBS[4]; Assistant Editor-In-Chief: Kristin Feeney, B.S. [5]

Overview

The cause of Total anomalous pulmonary venous connection has not been identified.

Causes

The cause of Total anomalous pulmonary venous connection has not been identified. To review risk factors for the development of total anomalous pulmonary venous connection, click here.

References

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Differentiating Total anomalous pulmonary venous connection from other Diseases

Differentiating Total anomalous pulmonary venous connection from other Diseases


Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Cafer Zorkun, M.D., Ph.D. [2]; Keri Shafer, M.D. [3]; Priyamvada Singh, MBBS[4]; Sahar Memar Montazerin, M.D.[5] Assistant Editor-In-Chief: Kristin Feeney, B.S. [6]

Overview

Total anomalous pulmonary venous connection should be differentiated from other cyanotic congenital heart diseases found in the pediatrics population. These disorders include tetralogy of Fallot, persistent truncus arteriosus, transposition of the great vessels, tricuspid atresia, trilogy of Fallot, and pentalogy of Fallot.

Differentiating Total anomalous pulmonary venous connection from other Diseases

Total anomalous pulmonary venous connection should be differentiated from other cyanotic congenital heart diseases found in the pediatrics population. These disorders include:

Disorders Etiology Clinical Presentation Laboratory Findings Electrocardiogram Findings Echocardiography Findings X-Ray Findings
Tetralogy of Fallot [1][2] Multifactorial

Echocardiography may show:

  • Residual VSD or ASD
  • RV outflow tract obstruction
  • Abnormal valvular anatomy
  • The boot-shaped heart appearance
  • Normal heart size
  • Pulmonary vascular marking may be normal or decreased
Total Anomalous Pulmonary Venous Connection [3][4][5] Multifactorial
  • Prominence of the pulmonary arteries
  • Mild enlargement of heart
  • The classic snowman sign is seen in supracardiac subtype
Tricuspid Atresia [6][7] Multifactorial
  • Respiratory difficulties as nasal flaring or muscle retractions
  • Cyanosis
  • Growth retradation
  • Tall P waves indicate atrial enlargement.
  • Frontal plane QRS axis may be leftward.
 Echocardiography may show
Transposition of the Great Arteries [8][9]

Multifactorial

Echocardiography may show:

  • The classic egg on string appearance
  • Pulmonary vascular marking may be normal or increased

Tetralogy of Fallot must be differentiated from other diseases that cause cyanosis:

Diseases Cyanosis Clinical manifestations Para-clinical findings Gold standard Additional findings
Symptoms Physical examination
Lab Findings Imaging
Peripheral Central Dyspnea Fever Chest pain Clubbing Peripheral edema Auscultation CBC ABG Electrolytes Other X-ray CT scan Other
Pulmonary diseases Airway disorder Severe croup[10] + – + +/- – – – Stridor Lymphocytosis Normal Normal – Steeple sign Normal Distended hypopharynx during inspiration Clinical findings Intercostal and subcostal retraction, Barking cough
Epiglottitis[11] + – + + – – – Stridor Leukocytosis with neutrophilia Normal Normal – Thumb sign Normal – Laryngoscopy Muffled voice, Drooling
Foreign body aspiration[12] + – + – +/- – – Decreased breath sounds, Wheezing Normal Normal Normal – Hyperinflation, Atelectasis, Objects Foreign body entrapment with edema or granulation tissue – Bronchoscopy Mediastinitis
Bacterial tracheitis[13] + – + + + – – Inspiratory stridor Leukocytosis Normal Normal Blood culture, Gram stain Irregular tracheal margin Normal Steeple sign (confusing) Laryngotracheobronchoscopy Brassy cough, Hoarseness
Sleep apnea[14] – + + – – – – Normal Polycythemia ↓O2, ↑CO2 Normal – Normal Normal Polysomnography Polysomnography Nightmares, Snoring
Chronic bronchitis[15] – + + +/- +/- + – Rales, crackles, Wheeze Leukocytosis ↓O2, ↑CO2, Respiratory acidosis Hypokalemia, Hypernatremia Gram stain of sputum Elongated heart, Flattened diaphragms, Prominent hilar vasculature Bronchial wall thickening with increased bronchovascular markings – HRCT Productive cough, Chest tightness
Atelectasis[16] – + + – + – – Localized diminished breath sounds, Wheeze Leukocytosis ↓O2, ↑CO2, Respiratory acidosis Normal – Localized increased opacity, Deviation toward the atelectasis Local crowding of pulmonary vessels and bronchi MRI for distinguishing obstructive from non-obstructive HRCT Cough, Shallow breathing
Parenchymal disorder Alveolitis[17] +/- + + + + +/- – Wheeze, Crackles Leukocytosis, Eosinophilia ↓O2, ↑CO2 Normal ↑ESR, ↑CRP Scattered opacities, Fine reticulation Homogeneous ground-glass opacity – HRCT PLUS Clinical findings Malaise, Chills, Headache
Pneumonia[18] – + + + + +/- – Rales, Crackles, Wheeze, Pleural friction rub Leukocytosis with neutrophilia Normal Hyponatremia – Entire lobe consolidated, Air bronchograms Focal ground-glass opacity – HRCT Tachycardia, Bradycardia (Legionella)
Asthma (Late)[19] – + + – +/- +/- – End expiratory wheeze Eosinophilia ↓O2, ↑CO2 Normal ↑ IgE Atelectasis Allergic bronchopulmonary aspergillosis, Bronchiectasis – Spirometry before and after bronchodilator Triad of asthma, nasal polyps, and rash is indicative of aspirin sensitivity.
Cystic fibrosis[20] + – +/- +/- + – Wheeze, Crackles Normal ↓O2, ↑CO2 Increased sweat chloride Sweat chloride test Hyperinflation, Nodules Peribronchial thickening, Bronchiectasis – Sweat chloride test Absent vas deferens
COPD

(Severe emphysema)[21]

+ – + +/- + +/- +/- Reduced breath sounds, Wheeze, Inspiratory crackles Polycythemia ↓O2, ↑CO2 Normal Alpha 1-antitrypsin test Elongated heart, Flattened diaphragms, Prominent hilar vasculature Bullae – HRCT Pulmonary hypertension, Right heart failure
Tuberculosis[22] – + + + + +/- – Reduced breath sounds, Wheeze, Inspiratory crackles Leukocytosis, ↑Lymphocyte ↓O2, ↑CO2 Hyponatremia, Hyperkalemia, Hypochloremia PPD, interferon-gamma release assay (IGRA) Dense, homogeneous parenchymal consolidation Nodules with low-density centers and rim enhancement Fluorodeoxyglucose positron emission tomography/CT (FDG PET/CT) Sputum culture, QuantiFERON-TB Gold (QFT) Loss of appetite, Night sweats
Pulmonary fibrosis[23] – + + – + + – Inspiratory crackles Anemia ↓O2, ↑CO2 Normal Matrix metalloproteinases (MMPs) Honeycombing Traction bronchiectasis,

Interlobular septal thickening

HRCT HRCT Fatigue, Weight loss
Pneumoconiosis[24] – + + – +/- +/- – End expiratory wheeze Leukocytosis, Anemia ↓O2, ↑CO2, Respiratory acidosis Hyper/Hypocalcemia,

Hypermagnesemia

– Small to large round nodular opacities Diffuse distribution of small nodules MRI and PET-CT scan CT/HRCT scan Tightness in the chest
Lung cancer[25] – + + – +/- + – Absence of breath sounds, Stridor, Wheezing Leukocytosis, Anemia ↓O2, ↑CO2 Hyponatremia ↑CRP, ↑ESR Pulmonary nodule or mass,

Mediastinal widening

Hilar lymphadenopathy

Massive lymphadenopathy, Direct mediastinal invasion MRI, PET-CT scan, Bronchoscopy Low dose computed tomography scan (LDCT) Weight loss, Loss of appetite
Acute respiratory distress syndrome[26] – + + +/- + – – Inspiratory crackles Leukopenia/Leukocytosis ↓O2, ↓CO2, Respiratory alkalosis Hyponatremia, Hyperkalemia ↑BNP, ↑Von Willebrand factor (VWF) Bilateral pulmonary infiltrates (patchy to diffuse) Emphysema, Pneumothorax and pneumomediastinum, Mediastinal lymphadenopathy Invasive Hemodynamic Monitoring (PCWP), Bronchoscopy Chest CT scan Tachypnea, Muscle weakness
Pulmonary vascular disorders Massive pulmonary embolism[27] + – + +/- + – +/- Reduced breath sounds, Crackles, Loud P2 Leukocytosis ↓O2, ↑CO2, Respiratory acidosis Normal D-dimer, BNP Fleischner sign, Hampton hump, Westermark sign, Pleural effusion Filling defects in the pulmonary vasculature Spiral CT pulmonary angiogram Spiral CT pulmonary angiogram Tachycardia, Shock, Pulmonary hypertension
Pulmonary arterio-venous malformation[28][29][30] – + + – + + – Pulmonary bruit Normal ↓O2, ↑CO2, Respiratory acidosis Normal – One or more rounded or multilobular opacities Connecting vessel in hilum Magnetic resonance angiography, Echocardiography Contrast enhanced magnetic resonance angiography Cerebral arteriovenous malformation
Pulmonary hypertension[31] – + + – +/- +/- – Tricuspid regurgitation murmur, Pulmonic insufficiency murmur Mild anemia ↓O2, ↑CO2 Hypernatremia ↑BNP, ↑ANP Right deviated cardiac apex,

Prominent pulmonary artery

Ill-defined nodules, Interlobular septal thickening CT pulmonary angiography (CTPA), MRA Cardiac catheterization (PCWP) Fatigue, Inability to exercise
Chest wall disorders Flail chest[32] + – + – + – – Normal Normal ↓O2, ↑CO2 Normal – ≥3 adjacent ribs with segmental fractures, >5 adjacent rib fractures Normal – CXR Bruises over chest
Pneumothorax[33] + – + – + – – Diminished breath sounds Normal ↓O2, ↑CO2 Normal – No lung marking on one side, Collapsed lung Loculated air in thoracic cavity M-mode ultrasonography Chest CT scan Tachypnea
Disease Peripheral Central Dyspnea Fever Chest pain Clubbing Peripheral edema Auscultation CBC ABG Electrolytes Other X-ray CT scan Other Gold standard Additional findings
Cardiac diseases Congenital disorders Atrioventricular canal defect[34] +/- + + – +/- +/- – Wheezing,Holosystolic or systolic ejection murmur Normal Normal Normal Pulse oximetry Cardiomegaly, Increased pulmonary vascular markings Normal Echocardiography, MRI Echocardiography Tachypnea, Lack of appetite, Pale skin color, Excessive sweating
Ebstein anomaly[35] – + +/- – +/- – – Loud S1 Normal Normal Normal Pulse oximetry Cardiomegaly, “Box shape” heart Apical displacement of the septal and posterior leaflets of the tricuspid valve “Atrialisation” of the right ventricle in MRI, Tricuspid regurgitation in echocardiography Echocardiography Fatigue, Palpitations
Tetralogy of Fallot[36] – + +/- – +/- – – Harsh systolic murmur Normal Normal Normal Pulse oximetry “Boot-shaped” heart with an upturned cardiac apex Aortopulmonary collateral vessels Peripheral pulmonary stenosis and atresia in echocardiography Echocardiography Fainting, Palpitation
Pulmonic stenosis[37] – + + – + +/- – Crescendo-decrescendo ejection murmur Schistocyte ↓O2 Normal Pulse oximetry Right ventricular hypertrophy, Dilated main pulmonary artery Stenotic segment, Post stenotic dilatation Severity of the stenosis by velocity encoded phase contrast (VEC) cine sequences Echocardiography Fainting, Palpitation
Total anomalous pulmonary venous connection[38] – + + – +/- +/- – Systolic murmur over the pulmonary area Normal ↓O2 Normal Pulse oximetry Snowman sign Anomalous venous return Blind ended left atrium with no connecting veins in echocardiography Echocardiography Pounding heart, Weak pulse, Extreme sleepiness
Transposition of the great vessels[39] – + + – +/- +/- – Diastolic and Systolic murmur Normal ↓O2 Normal Pulse oximetry Cardiomegaly with narrow superior mediastinum (egg on a string sign) Abnormal great vessel anatomy Flow dynamics on Steady-state free precession (SSFP) MRI Echocardiography Lack of appetite, Poor weight gain
Truncus arteriosus[40] – + +/- – +/- +/- – Holosystolic or ejection type murmur Normal ↓O2 Normal Pulse oximetry Moderate cardiomegaly, pulmonary plethora, Widened mediastinum Single cardiac trunk Anomalous anatomy in MRI Echocardiography Fatigue, Sweating, Pale or cool skin
Patent ductus arteriosus[41] – + +/- – +/- +/- – Continuous machinery murmur Normal ↓O2, ↑CO2 Normal Pulse oximetry Cardiomegaly, Aortopulmonary window obsecuration Tortuous morphology of ductus (Krichenko classification) Spiral CT pulmonary angiogram Echocardiography Failure to thrive, Respiratory distress
Acquired disorders Heart failure[42] + – + +/- + – + Coarse crackles, S3 Anemia ↓O2, ↑CO2 Hyponatremia, Hypokalemia, Hypomagnesemia Elevated BNP Pleural effusion, Cardiomegaly Kerley B lines Normal Radioisotope scan Echocardiography Generalized edema, Hepatomegaly
Valvular heart disease[43] + – + – – – + Murmurs Schistocyte Normal Normal Hyperthyroidism Valve calcification Normal Valvular dysfunction in echocardiography Echocardiography Syncope, Palpitation
Myocardial infarction[44] – + + +/- + – – S3 and S4 Normal Normal Hyponatremia, Hypokalemia Elevated troponin I and CKMB Rolling out other causes Coronary luminal narrowing in CT perfusion scan Wall motion abnormality in echocardiography Cardiac troponin I Dizziness, Fatigue, Lightheadedness, Cold sweat
Cardiogenic shock[45] +/- + + – – – + Muffled heart sound Anemia ↓O2, ↑CO2 Hyperkalemia ↓PaO2 in pulse oximetry Normal Normal Echocardiography Clinical findings Tachypnea, Palpitation, Hypotension, Weak pulse
Cardiomyopathy[46] – + + – + + + S3 and S4 Lymphocytosis ↓O2, ↑CO2 Normal ↓PaO2 in pulse oximetry Enlarged left ventricle and atria, Pulmonary edema Normal MRI Endomyocardial biopsy Arrhythmia, Bloating
Heart tumors[47] – + + – +/- +/- +/- Early diastolic tumor plop Normal Normal Normal Mild ↓PaO2 in pulse oximetry Calcification in lateral view Intracardiac mass Echocardiography Histologic diagnosis (biopsy) Syncope, Weight loss
Mitral Stenosis[48] – + + – +/- +/- +/- Diastolic murmur Normal Normal Normal Mild ↓PaO2 in pulse oximetry Left atrial enlargement, Mitral annular calcification Secondary pulmonary hemosiderosis Velocity-encoded cine-magnetic resonance imaging (VEC-MRI) Echocardiography Dizziness, Hemoptysis
Vascular disease Arterial disorders Acrocyanosis[49] + – +/- – – + – Normal Anemia, Leukocytosis ↓O2, ↑CO2 Hypercalcemia ↓PaO2 in pulse oximetry Normal Normal – Clinical findings Brittle nails, Telangiectasia
Arterial embolism[50] + – + – + +/- – Normal Normal Normal Hyperkalemia ↓PaO2 in pulse oximetry Normal Normal Transesophageal echocardiography (TEE) Clinical findings Headache, Decreased sensation
Raynaud’s Phenomenon[51] + – – – – + – Normal Polycythemia ↓O2 Normal Mild ↓PaO2 in pulse oximetry Normal Normal – Clinical findings Sensitivity to cold, Decreased sensation
Venous disorders Superior vena cava obstruction[52] + – +/- – +/- +/- + Normal Polycythemia ↓O2 Normal ↓PaO2 in pulse oximetry Superior mediastinal widening, Right hilar prominence Thrombosis, Mediastinal mass or lymphadenopathy, Associated lung mass – Chest CT scan Headache,

Facial swelling

Venous stasis[53] + – – – – +/- + Normal Polycythemia ↓O2 Hypercalcemia Normal Normal Normal Color-flow duplex ultrasound Color-flow duplex ultrasound Leg swelling, Pain during walking,

Leg ulcers

Disease Peripheral Central Dyspnea Fever Chest pain Clubbing Peripheral edema Auscultation CBC ABG Electrolytes Other X-ray CT scan Other Gold standard Additional findings
Hematologic diseases Methemoglobinemia[54] – + + +/- + – – Wheezing Anemia, Methemoglobinemia Normal PaO2, ↑SaO2, “Saturation gap” Normal ↓SaO2 in pulse oximetry Normal Normal Echocardiography for ruling out other causes Co-oximetry, ABG paired with pulse oximetry. Serum methemoglobin levels Headache, Altered mental status, Delirium, Seizure, Coma
Polycythemia[55] + + + +/- +/- + – Normal ↑RBC, ↑WBC, ↑HGB, ↑Plt ↓O2 Hyperkalemia ↑Leukocyte alkaline phosphatase, ↑Ferritin, ↑Erythropoietin AVM, COPD, pulmonary hypertension Normal Abdominal ultrasound or renal vascular studies for ruling out renal artery stenosis RBC mass (RCM) and plasma volume measurement Itchiness, Headache. Dizziness. Blurred vision
Neurological disease Breath-holding spells[56] – + + – +/- – – Wheezing Hypochromic microcytic anemia ↓O2 Hypocalcemia, Hypokalemia Iron deficiency Ruling out foreign body aspiration Normal EEG monitoring Clinical findings Fainting, Twitching muscles, Seizure
Seizure[57] – + + +/- – – – Normal Normal ↓O2, ↑CO2 Hyponatremia, Hypo/Hypercalcemia ↑CPK, ↑LDH, Normal Normal EEG EEG Fainting, Tonic-clonic movements
Coma[58] – + – – – – +/- Wheezing Normal ↓O2, ↑CO2 Normal – Underlying disease Underlying disease – Glasgow Coma Scale (GCS) Depressed brainstem reflexes, Agonal breathing
Head trauma[59] – + + – – – – Normal Normal ↓O2, ↑CO2 Normal – Skull fracture Intracranial hemorrhage MRI CT scan Nausea, Hypertension, Bradycardia,Tachypnea
Miscellaneous High altitude exposure[60] +/- + + – +/- + – Wheezing Polycythemia ↓O2, ↓CO2, Respiratory alkalosis Hyperphosphatemia, Hypercalcemia, Hyponatremia, Hypokalemia, Hypomagnesemia Decreased bicarbonate Central interstitial edema Pulmonary consolidation – Hypoxic challenge test Dizziness, Coma, Death
Septic shock[61] + – + + +/- – – Rales, crackles, Wheeze, Pleural friction rub Leukocytosis with neutrophilia ↓O2, ↑CO2, Metabolic acidosis Hyperkalemia ↑ESR, ↑CRP Consolidation Pulmonary infiltration Echocardiography Blood culture Chills, Hypothermia, Loss of consciousness
Smoke inhalation[62] +/- + + – + – – Wheezing Anemia, Carboxyhemoglobin ↓O2, ↑CO2, Respiratory acidosis Normal CO-oximetry Diffuse opacities Pulmonary infiltration Pulmonary function testing Bronchoscopy Cough, Hoarseness, Hemoptysis, Headache, Fainting
Cold exposure[63] + – +/- – – – – Normal Leukopenia, ↑RBC ↓O2, ↑CO2, Metabolic acidosis Hypokalemia, Hypocalcemia Hyperglycemia, ↑CK Normal Normal – Clinical findings Confusion, Tachycardia/Bradycardia, Coma

References

  1. ↑ Morris, Douglas C.; Felner, Joel M.; Schlant, Robert C.; Franch, Robert H. (1975). “Echocardiographic diagnosis of tetralogy of Fallot”. The American Journal of Cardiology. 36 (7): 908–913. doi:10.1016/0002-9149(75)90081-8. ISSN 0002-9149.
  2. ↑ Kothari SS (October 1992). “Mechanism of cyanotic spells in tetralogy of Fallot–the missing link?”. Int. J. Cardiol. 37 (1): 1–5. doi:10.1016/0167-5273(92)90125-m. PMID 1428277.
  3. ↑ Zhang, Ziming; Zhang, Li; Xie, Feng; Wang, Bing; Sun, Zhengxing; Kong, Shuangshuang; Wang, Xinfang; Dong, Nianguo; Wang, Guohua; Lv, Qing; Li, Yuman; Li, Ling; Xie, Mingxing (2016). “Echocardiographic diagnosis of anomalous pulmonary venous connections”. Medicine. 95 (44): e5389. doi:10.1097/MD.0000000000005389. ISSN 0025-7974.
  4. ↑ Chen JT (October 1979). “Radiologic demonstration of anomalous pulmonary venous connection and its clinical significance”. CRC Crit Rev Diagn Imaging. 11 (4): 383–422. PMID 389559.
  5. ↑ Gathman, Gary E.; Nadas, Alexander S. (1970). “Total Anomalous Pulmonary Venous Connection”. Circulation. 42 (1): 143–154. doi:10.1161/01.CIR.42.1.143. ISSN 0009-7322.
  6. ↑ Beppu, S; Nimura, Y; Tamai, M; Nagata, S; Matsuo, H; Kawashima, Y; Kozuka, T; Sakakibara, H (1978). “Two-dimensional echocardiography in diagnosing tricuspid atresia. Differentiation from other hypoplastic right heart syndromes and common atrioventricular canal”. Heart. 40 (10): 1174–1183. doi:10.1136/hrt.40.10.1174. ISSN 1355-6037.
  7. ↑ Thiene G, Anderson RH (1981). “The clinical morphology of tricuspid atresia. Atresia of the right atrioventricular valve”. G Ital Cardiol. 11 (12): 1845–59. PMID 7049815.
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  9. ↑ Warnes, Carole A. (2006). “Transposition of the Great Arteries”. Circulation. 114 (24): 2699–2709. doi:10.1161/CIRCULATIONAHA.105.592352. ISSN 0009-7322.
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  17. ↑ Lee JS, Im JG, Ahn JM, Kim YM, Han MC (August 1992). “Fibrosing alveolitis: prognostic implication of ground-glass attenuation at high-resolution CT”. Radiology. 184 (2): 451–4. doi:10.1148/radiology.184.2.1620846. PMID 1620846.
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  20. ↑ “Cystic fibrosis – Genetics Home Reference”.
  21. ↑ Qureshi H, Sharafkhaneh A, Hanania NA (2014). “Chronic obstructive pulmonary disease exacerbations: latest evidence and clinical implications”. Ther Adv Chronic Dis. 5 (5): 212–27. doi:10.1177/2040622314532862. PMC 4131503. PMID 25177479.
  22. ↑ Olalekan, Adebimpe Wasiu; Oluwaseun, Faremi Ayodeji; Oladele, Hassan Abdul-Wasiu; Akeem, Adeyemi Damilare (2015). “Evaluation of electrolyte imbalance among tuberculosis patients receiving treatments in Southwestern Nigeria”. Alexandria Journal of Medicine. 51 (3): 255–260. doi:10.1016/j.ajme.2014.10.003. ISSN 2090-5068.
  23. ↑ Shaw J, Marshall T, Morris H, Hayton C, Chaudhuri N (2017). “Idiopathic pulmonary fibrosis: a holistic approach to disease management in the antifibrotic age”. J Thorac Dis. 9 (11): 4700–4707. doi:10.21037/jtd.2017.10.111. PMC 5721024. PMID 29268540.
  24. ↑ Yen CM, Lin CL, Lin MC, Chen HY, Lu NH, Kao CH (2016). “Pneumoconiosis increases the risk of congestive heart failure: A nationwide population-based cohort study”. Medicine (Baltimore). 95 (25): e3972. doi:10.1097/MD.0000000000003972. PMC 4998335. PMID 27336897.
  25. ↑ Hartwig MG, D’Amico TA (June 2010). “Thoracoscopic lobectomy: the gold standard for early-stage lung cancer?”. Ann. Thorac. Surg. 89 (6): S2098–101. doi:10.1016/j.athoracsur.2010.02.102. PMID 20493989.
  26. ↑ Ochiai R (2015). “Mechanical ventilation of acute respiratory distress syndrome”. J Intensive Care. 3 (1): 25. doi:10.1186/s40560-015-0091-6. PMC 4456061. PMID 26045965.
  27. ↑ Bĕlohlávek J, Dytrych V, Linhart A (2013). “Pulmonary embolism, part I: Epidemiology, risk factors and risk stratification, pathophysiology, clinical presentation, diagnosis and nonthrombotic pulmonary embolism”. Exp Clin Cardiol. 18 (2): 129–38. PMC 3718593. PMID 23940438.
  28. ↑ Khurshid I, Downie GH (2002). “Pulmonary arteriovenous malformation”. Postgrad Med J. 78 (918): 191–7. PMC 1742331. PMID 11930021.
  29. ↑ Doshi HM, Robinson S, Chalhoub T, Jack S, Denison A, Gibson G (2009). “Massive spontaneous hemothorax during the immediate postpartum period”. Tex Heart Inst J. 36 (3): 247–9. PMC 2696501. PMID 19568398.
  30. ↑ Chanatry BJ (1992). “Acute hemothorax owing to pulmonary arteriovenous malformation in pregnancy”. Anesth. Analg. 74 (4): 613–5. PMID 1554132.
  31. ↑ Hoeper MM, Ghofrani HA, Grünig E, Klose H, Olschewski H, Rosenkranz S (2017). “Pulmonary Hypertension”. Dtsch Arztebl Int. 114 (5): 73–84. doi:10.3238/arztebl.2017.0073. PMC 5331483. PMID 28241922.
  32. ↑ Pettiford BL, Luketich JD, Landreneau RJ (February 2007). “The management of flail chest”. Thorac Surg Clin. 17 (1): 25–33. doi:10.1016/j.thorsurg.2007.02.005. PMID 17650694.
  33. ↑ Luh SP (2010). “Review: Diagnosis and treatment of primary spontaneous pneumothorax”. J Zhejiang Univ Sci B. 11 (10): 735–44. doi:10.1631/jzus.B1000131. PMC 2950234. PMID 20872980.
  34. ↑ Macris MP, Ott DA, Cooley DA (1992). “Complete atrioventricular canal defect: surgical considerations”. Tex Heart Inst J. 19 (3): 239–43. PMC 326195. PMID 15227445.
  35. ↑ Safi LM, Liberthson RR, Bhatt A (September 2016). “Current Management of Ebstein’s Anomaly in the Adult”. Curr Treat Options Cardiovasc Med. 18 (9): 56. doi:10.1007/s11936-016-0478-2. PMID 27439413.
  36. ↑ Bailliard F, Anderson RH (2009). “Tetralogy of Fallot”. Orphanet J Rare Dis. 4: 2. doi:10.1186/1750-1172-4-2. PMC 2651859. PMID 19144126.
  37. ↑ Yoo BW, Park HK (2013). “Pulmonary stenosis and pulmonary regurgitation: both ends of the spectrum in residual hemodynamic impairment after tetralogy of Fallot repair”. Korean J Pediatr. 56 (6): 235–41. doi:10.3345/kjp.2013.56.6.235. PMC 3693041. PMID 23807889.
  38. ↑ Stein P (March 2007). “Total anomalous pulmonary venous connection”. AORN J. 85 (3): 509–20, quiz 521–4. doi:10.1016/S0001-2092(07)60123-9. PMID 17352891.
  39. ↑ Martins P, Castela E (2008). “Transposition of the great arteries”. Orphanet J Rare Dis. 3: 27. doi:10.1186/1750-1172-3-27. PMC 2577629. PMID 18851735.
  40. ↑ Van Praagh R (1987). “Truncus arteriosus: what is it really and how should it be classified?”. Eur J Cardiothorac Surg. 1 (2): 65–70. PMID 2856609.
  41. ↑ “Patent Ductus Arteriosus – National Library of Medicine – PubMed Health”.
  42. ↑ Inamdar AA, Inamdar AC (2016). “Heart Failure: Diagnosis, Management and Utilization”. J Clin Med. 5 (7). doi:10.3390/jcm5070062. PMC 4961993. PMID 27367736.
  43. ↑ Maganti K, Rigolin VH, Sarano ME, Bonow RO (2010). “Valvular heart disease: diagnosis and management”. Mayo Clin Proc. 85 (5): 483–500. doi:10.4065/mcp.2009.0706. PMC 2861980. PMID 20435842.
  44. ↑ “Myocardial Infarction (Heart Attack): Symptoms – National Library of Medicine – PubMed Health”.
  45. ↑ Werdan K, Ruß M, Buerke M, Delle-Karth G, Geppert A, Schöndube FA; et al. (2012). “Cardiogenic shock due to myocardial infarction: diagnosis, monitoring and treatment: a German-Austrian S3 Guideline”. Dtsch Arztebl Int. 109 (19): 343–51. doi:10.3238/arztebl.2012.0343. PMC 3364528. PMID 22675405.
  46. ↑ Marian AJ, Braunwald E (September 2017). “Hypertrophic Cardiomyopathy: Genetics, Pathogenesis, Clinical Manifestations, Diagnosis, and Therapy”. Circ. Res. 121 (7): 749–770. doi:10.1161/CIRCRESAHA.117.311059. PMID 28912181.
  47. ↑ Hoffmeier A, Sindermann JR, Scheld HH, Martens S (2014). “Cardiac tumors–diagnosis and surgical treatment”. Dtsch Arztebl Int. 111 (12): 205–11. doi:10.3238/arztebl.2014.0205. PMC 3983698. PMID 24717305.
  48. ↑ Kannan M, Vijayanand G (2010). “Mitral stenosis and pregnancy: Current concepts in anaesthetic practice”. Indian J Anaesth. 54 (5): 439–44. doi:10.4103/0019-5049.71043. PMC 2991654. PMID 21189882.
  49. ↑ Das S, Maiti A (2013). “Acrocyanosis: an overview”. Indian J Dermatol. 58 (6): 417–20. doi:10.4103/0019-5154.119946. PMC 3827510. PMID 24249890.
  50. ↑ Lyaker MR, Tulman DB, Dimitrova GT, Pin RH, Papadimos TJ (2013). “Arterial embolism”. Int J Crit Illn Inj Sci. 3 (1): 77–87. doi:10.4103/2229-5151.109429. PMC 3665125. PMID 23724391.
  51. ↑ Block JA, Sequeira W (June 2001). “Raynaud’s phenomenon”. Lancet. 357 (9273): 2042–8. doi:10.1016/S0140-6736(00)05118-7. PMID 11438158.
  52. ↑ Cohen R, Mena D, Carbajal-Mendoza R, Matos N, Karki N (2008). “Superior vena cava syndrome: A medical emergency?”. Int J Angiol. 17 (1): 43–6. PMC 2728369. PMID 22477372.
  53. ↑ Fan CM (2005). “Venous pathophysiology”. Semin Intervent Radiol. 22 (3): 157–61. doi:10.1055/s-2005-921949. PMC 3036287. PMID 21326688.
  54. ↑ Ashurst J, Wasson M (July 2011). “Methemoglobinemia: a systematic review of the pathophysiology, detection, and treatment”. Del Med J. 83 (7): 203–8. PMID 21954509.
  55. ↑ Spivak JL (February 2002). “The optimal management of polycythaemia vera”. Br. J. Haematol. 116 (2): 243–54. PMID 11841424.
  56. ↑ Goldman RD (2015). “Breath-holding spells in infants”. Can Fam Physician. 61 (2): 149–50. PMC 4325862. PMID 25676645.
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  62. ↑ Rehberg S, Maybauer MO, Enkhbaatar P, Maybauer DM, Yamamoto Y, Traber DL (2009). “Pathophysiology, management and treatment of smoke inhalation injury”. Expert Rev Respir Med. 3 (3): 283–297. doi:10.1586/ERS.09.21. PMC 2722076. PMID 20161170.
  63. ↑ Marriott, Bernadette (1996). Nutritional needs in cold and in high-altitude environments : applications for military personnel in field operations. Washington, D.C: National Academy Press. ISBN 0-309-05484-2.

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Epidemiology and Demographics

Epidemiology and Demographics


Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Cafer Zorkun, M.D., Ph.D. [2]; Priyamvada Singh, MBBS [3]; Assistant Editor-In-Chief: Kristin Feeney, B.S. [4]

Overview

Total anomalous pulmonary venous connection is a rare cyanotic congenital heart disease and occurs in 6 to 12 per 100,000 live births. Total anomalous pulmonary venous connection affects both gender equally.

Epidemiology and Demographics

Incidence

Prevalence

  • There is no available information regarding the prevalence of total anomalous pulmonary venous connection.

Gender

  • Total anomalous pulmonary venous connection affects both gender equally.

References

  1. ↑ Reller MD, Strickland MJ, Riehle-Colarusso T, Mahle WT, Correa A (2008). “Prevalence of congenital heart defects in metropolitan Atlanta, 1998-2005”. J Pediatr. 153 (6): 807–13. doi:10.1016/j.jpeds.2008.05.059. PMC 2613036. PMID 18657826.
  2. ↑ Correa-Villaseñor, Adolfo; Ferencz, Charlotte; Boughman, Joann A.; Neill, Catherine A. (1991). “Total anomalous pulmonary venous return: Familial and environmental factors”. Teratology. 44 (4): 415–428. doi:10.1002/tera.1420440408. ISSN 0040-3709.
  3. ↑ Itoi, Toshiyuki (2013). “Stenting as a possible new therapeutic strategy to the obstructed TAPVC”. Journal of Cardiology Cases. 8 (2): e93–e94. doi:10.1016/j.jccase.2013.05.001. ISSN 1878-5409.

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Risk Factors

Risk Factors


Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Cafer Zorkun, M.D., Ph.D. [2]; Keri Shafer, M.D. [3]; Priyamvada Singh, MBBS[4]; Assistant Editor-In-Chief: Kristin Feeney, B.S. [5]

Overview

There are certain factors that increase the risk of congenital heart diseases, including maternal obesity, medication during pregnancy, alcohol and drugs, rubella during pregnancy, diabetes during pregnancy, and genetic factors.

Risk Factors

Risk factors during congenital heart diseases are:[1][2][3][4][5]

References

  1. ↑ Alvarado-Terrones, Evelyn G.; Perea-Cabrera, Maryangel; Klünder-Klünder, Miguel; Segura-Stanford, Begoña; Erdmenger-Orellana, Julio R.; Lopez-Yañez Blanco, Arturo; Hernández-Carbajal, Elizabeth; Granados Riverón, Javier T.; Mejía-Marín, Leonardo J.; Balderrabano-Saucedo, Norma A.; Contreras-Ramos, Alejandra; Díaz-Rosas, Guadalupe; Sánchez-Urbina, Rocío (2018). “Maternal Obesity as a Risk Factor for the Development of Total Anomalous Pulmonary Venous Connection in Their Offspring”. Archives of Medical Research. 49 (2): 109–113. doi:10.1016/j.arcmed.2018.06.001. ISSN 0188-4409.
  2. ↑ Correa-Villaseñor, Adolfo; Ferencz, Charlotte; Boughman, Joann A.; Neill, Catherine A. (1991). “Total anomalous pulmonary venous return: Familial and environmental factors”. Teratology. 44 (4): 415–428. doi:10.1002/tera.1420440408. ISSN 0040-3709.
  3. ↑ Phelan, K.; McDermid, H.E. (2011). “The 22q13.3 Deletion Syndrome (Phelan-McDermid Syndrome)”. Molecular Syndromology. doi:10.1159/000334260. ISSN 1661-8777.
  4. ↑ Bleyl, Steven B.; Saijoh, Yukio; Bax, Noortje A.M.; Gittenberger-de Groot, Adriana C.; Wisse, Lambertus J.; Chapman, Susan C.; Hunter, Jennifer; Shiratori, Hidetaka; Hamada, Hiroshi; Yamada, Shigehito; Shiota, Kohei; Klewer, Scott E.; Leppert, Mark F.; Schoenwolf, Gary C. (2010). “Dysregulation of the PDGFRA gene causes inflow tract anomalies including TAPVR: integrating evidence from human genetics and model organisms”. Human Molecular Genetics. 19 (7): 1286–1301. doi:10.1093/hmg/ddq005. ISSN 0964-6906.
  5. ↑ Cluver, C.; Meyer, R.; Odendaal, H.; Geerts, L. (2013). “Congenital rubella with agenesis of the inferior cerebellar vermis and total anomalous pulmonary venous drainage”. Ultrasound in Obstetrics & Gynecology. 42 (2): 235–237. doi:10.1002/uog.12399. ISSN 0960-7692.

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Screening

Screening


Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Cafer Zorkun, M.D., Ph.D. [2]; Keri Shafer, M.D. [3] Priyamvada Singh, MBBS [4]; Assistant Editor-In-Chief: Kristin Feeney, B.S. [5]

Overview

There is insufficient evidence to recommend routine screening for total anomalous pulmonary venous connection.

Screening

There is insufficient evidence to recommend routine screening for total anomalous pulmonary venous connection.

References

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Natural History, Complications and Prognosis

Natural History, Complications and Prognosis


Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Cafer Zorkun, M.D., Ph.D. [2]; Keri Shafer, M.D. [3] Priyamvada Singh, MBBS [4]; Raviteja Guddeti, M.B.B.S. [5]; Assistant Editor-In-Chief: Kristin Feeney, B.S. [6]

Overview

The natural history of untreated patients of TAPVC is not very favorable. It depends on the type of obstruction and amount of shunting across the atrium.

Natural History

The natural history of untreated patients of total anomalous pulmonary venous connection depends on:

  • Type of obstruction (unobstructed, obstructed)
  • Type of anatomic variant(cardiac, infracardiac or supracardiac)
  • Amount of right-to-left shunting

Untreated patients:[1]

  • Patients with severe obstruction may not be able to survive beyond few months of life.
  • 8 out of 10 patients with small interatrial connections and obstruction die as an infant.
  • The presentation of patients with unobstructed TAPVC and large interatrial shunting may vary from asymptomatic to symptoms due to progressive right heart failure.

Complications

Complications of TAPVC include:

Long-term complications include:

Prognosis

Prognosis of total anomalous pulmonary venous depends on whether the surgery has been done with the patient. Without surgery, death may occur by age one in babies with more severe defects.

References

  1. ↑ BURROUGHS JT, EDWARDS JE (1960). “Total anomalous pulmonary venous connection”. Am Heart J. 59: 913–31. PMID 13806293.

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Diagnosis

Diagnosis

History and Symptoms | Physical Examination | Laboratory Findings | Electrocardiogram | Chest X Ray | MRI | CT | Echocardiography | Other Imaging Findings | Other Diagnostic Studies

Treatment

Treatment

Medical Therapy | Surgery | Prevention

Case Studies

Case Studies

Case #1

References

References

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