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[Atrial septal defect with pulmonary hypertension in infancy (author's transl)]
Insights
This study reports four pediatric cases of large atrial septal defects (ASD) causing pulmonary hypertension and heart failure. Early diagnosis is challenging, but surgical correction offers good long-term outcomes for surviving patients.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Pulmonary Hypertension
Context:
- Atrial septal defects (ASD) are common congenital heart anomalies.
- Ostium secundum ASDs can rarely lead to severe pulmonary hypertension and congestive heart failure in infants.
- Accurate pre-operative diagnosis in young children can be challenging.
Purpose:
- To report cases of severe pulmonary hypertension and congestive heart failure in infants due to large ostium secundum atrial septal defects.
- To highlight diagnostic challenges and discuss the management and outcomes of surgical correction.
- To review the literature on this rare presentation of ASD.
Summary:
- Four infants under two years old presented with large ostium secundum ASDs, severe pulmonary hypertension (systolic pressure >50 mm Hg), and congestive heart failure.
- Pre-operative diagnosis was accurate in only two cases; misdiagnoses included partial anomalous pulmonary venous connection and exclusion of ventricular septal defect.
- One patient died post-catheterization; the other three underwent successful surgical correction with excellent long-term results (asymptomatic after 4.5 years average follow-up).
Impact:
- This case series underscores the rarity and diagnostic difficulties of severe pulmonary hypertension secondary to large ASDs in infants.
- It emphasizes the importance of considering ASD in infants with unexplained heart failure and pulmonary hypertension.
- Successful surgical intervention can lead to excellent long-term outcomes, highlighting the curability of this condition.
Abstract:
Four cases of atrial septal defect, "ostium secundum", associated with pulmonary hypertension and congestive heart failure in children under the age of two years are reported. A correct diagnosis could be made before surgery in only two of the case. In one case partial anomalous pulmonary venous connection was incorrectly diagnosed, while ventricular septal defect could not be excluded in the fourth case. The systolic pressure in the pulmonary artery was higher than 50 mm. Hg in all cases. One patient died after catheterization, and total surgical correction was performed in the other three cases, with good results. In all of them, a large atrial septal defect could be seen. There was no evidence of any other associated anomaly. After an average follow up of 4.5 years the three patients that where operated remain asymptomatic. The rarity of this entity, the difficulties in making a correct diagnosis and several pathogenetic aspects of this abnormality are discussed on the light of these and other cases previously reported in the literature.