Multiple ventricular septal defects and severe tricuspid regurgitation in an infant - a case report and surgical dilemma

BACKGROUND: Ventricular septal defect is the most prevalent form of congenital heart disease, occurring as isolated lesion in approximately 20% of cases and as part of more complex malformations in up to 40%. Multiple ventricular septal defects are usually associated with "Swiss Cheese" variant characterized by multiple perforations throughout the muscular septum, often involving the apical, mid-muscular, and anterior regions, which creates a complex network of left-to-right shunts that are technically difficult to identify and close individually. Presence of severe tricuspid regurgitation contributes to risks related to poor prognosis. An infant with congestive heart failure, severe tricuspid regurgitation, large left-to-right shunting and elevated right ventricular pressure requires a critical decision-making process for surgical management due to higher risks and poor outcome. CASE PRESENTATION: We are presenting herewith a six-month-old infant with Holt-Oram syndrome, who presented with classic signs of congestive heart failure, tachypnoea, poor weight gain and hepatomegaly, all indicative of systemic and pulmonary venous congestion. Chest x-ray and echocardiography confirmed the diagnosis. Despite aggressive medical management the patient's condition continued to deteriorate and required surgical intervention. The traditional management paradigm for such infants involves a choice between palliative pulmonary artery banding or surgical closure. In this case, both options presented significant risks and were deemed suboptimal. Therefore, to avoid risks, we opted a minimal invasive perventricular device closure of the defects with success. CONCLUSIONS: Children with severe tricuspid regurgitation, right ventricular failure, Swiss cheese morphology, multiple ventricular septal defects, genetic disorders and low weight may be considered for minimal invasive approaches including perventricular device closure of the ventricular septal defects whenever feasible to avoid operative risks and offer better outcomes.