Endocardial cushion defect natural history, complications and prognosis

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Aditya Ganti M.B.B.S. [2]

Overview

If left untreated, majority of patients with endocardial cushion defect may progress to develop life threatening conditions. Common complications of endocardial cushion defect include dilatation of heart, pulmonary hypertension, respiratory tract infections , and heart failure. Surgical mortality rate of patients with partial endocardial cushion defect is approximately 0.6%. For complete cushion defect the surgical mortality rate is 2.5-9%. Prognosis of endocardial cushion defect is generally good with treatment. However, some children might develop valvular and rhythm disorders after surgical correction.

Natural History, Complications, and Prognosis

Natural History

If left untreated, majority of patients with endocardial cushion defect may progress to develop life threatening heart failure and pulmonary hypertension due to excessive pulmonary blood flow.

Complications

Common complications of endocardial cushion defect include:^[1]^[2]

Prognosis

Prognosis of endocardial cushion defect is generally good with treatment. However, some children might develop valvular and rhythm disorders after surgical correction.^[3]
Prognositic factors include:
- Degree of preoperative pulmonary vascular disease
- Amount of residual AV valve regurgitation
Protected pulmonary vascular bed with mild AV regurtiation is associated with good outcome, while the contrary holds the bad prognosis.
Surgical mortality rate of patients with partial endocardial cushion defect is approximately 0.6%. For complete cushion defect the surgical mortality rate is 2.5-9%

References

↑ Gowda RM, Ansari AW, Khan IA (May 2003). “Complete endocardial cushion defect (complete atrioventricular canal) manifested in adult life by Streptococcus mitis endocarditis of the common atrioventricular valve”. Int. J. Cardiol. 89 (1): 109–10. doi:10.1016/s0167-5273(02)00459-x. PMID 12727016.
↑ Yıldırım G, Gungorduk K, Yazıcıoğlu F, Gul A, Cakar F, Celikkol O, Ceylan Y (2009). “Prenatal diagnosis of complete atrioventricular septal defect: perinatal and neonatal outcomes”. Obstet Gynecol Int. 2009: 958496. doi:10.1155/2009/958496. PMC 2778174. PMID 19960047.
↑ Maltret A, Moura C, Le Bidois J, Fermont L, Bajolle F, Stos B, Azancot A, Bonnet D (May 2007). “[Prognosis of atrioventricular canal in euploid foetus without abnormality of atrial situs]”. Arch Mal Coeur Vaiss (in French). 100 (5): 411–5. PMID 17646766.

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[pmid12727016-1] Gowda RM, Ansari AW, Khan IA (May 2003). “Complete endocardial cushion defect (complete atrioventricular canal) manifested in adult life by Streptococcus mitis endocarditis of the common atrioventricular valve”. Int. J. Cardiol. 89 (1): 109–10. doi:10.1016/s0167-5273(02)00459-x. PMID 12727016.

[pmid19960047-2] Yıldırım G, Gungorduk K, Yazıcıoğlu F, Gul A, Cakar F, Celikkol O, Ceylan Y (2009). “Prenatal diagnosis of complete atrioventricular septal defect: perinatal and neonatal outcomes”. Obstet Gynecol Int. 2009: 958496. doi:10.1155/2009/958496. PMC 2778174. PMID 19960047.

[pmid17646766-3] Maltret A, Moura C, Le Bidois J, Fermont L, Bajolle F, Stos B, Azancot A, Bonnet D (May 2007). “[Prognosis of atrioventricular canal in euploid foetus without abnormality of atrial situs]”. Arch Mal Coeur Vaiss (in French). 100 (5): 411–5. PMID 17646766.

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