What is the best treatment for a 4-year-old child with infracardiac total anomalous pulmonary venous connection (TAPVC) with atrial septal defect (ASD) and incomplete patent ductus arteriosus (PDA) and patent ductus venosus?

From the Guidelines

The best treatment for a 4-year-old child with infracardiac Total Anomalous Pulmonary Venous Connection (TAPVC), Atrial Septal Defect (ASD), incomplete Patent Ductus Arteriosus (PDA), and patent ductus venosus is surgical correction, as it is the most effective way to reduce morbidity, mortality, and improve quality of life. This complex congenital heart condition requires complete surgical repair rather than medical management alone. The surgery involves redirecting the pulmonary veins to connect properly to the left atrium, closing the ASD, and ligating the PDA and patent ductus venosus.

Pre-operative Management

Pre-operative management includes:

• Diuretics like furosemide (1-2 mg/kg/dose) to manage pulmonary congestion, as recommended by 1
• Possibly prostaglandin E1 (starting at 0.05-0.1 mcg/kg/min) if the child is dependent on the PDA for pulmonary blood flow

Post-operative Care

Post-operative care will include:

• ICU monitoring
• Ventilatory support
• Inotropic medications like milrinone (0.25-0.75 mcg/kg/min) or dopamine (5-10 mcg/kg/min)
• Continued diuretic therapy According to 1, cardiac catheterization should be considered to measure PVRI and to determine operability, and repair should be considered if PVRI is <6 WU·m or PVR/SVR <0.3 at baseline.

Long-term Follow-up

Long-term follow-up with a pediatric cardiologist is essential to monitor for potential complications such as pulmonary vein stenosis or pulmonary hypertension. Surgical correction is necessary because this condition causes oxygenated blood from the lungs to return to the right side of the heart instead of the left, creating a significant left-to-right shunt that leads to right heart volume overload and pulmonary congestion.

From the Research

Treatment Options for Infra-Cardiac TAPVC with ASD and Incomplete PDA

• The treatment for a 4-year-old baby with infra-cardiac Total Anomalous Pulmonary Venous Connection (TAPVC) with Atrial Septal Defect (ASD) and incomplete Patent Ductus Arteriosus (PDA) and patent ductus venous is complex and requires a multidisciplinary approach 2, 3.
• Echocardiography plays a crucial role in the diagnosis and management of TAPVC, as it can accurately determine the drainage sites and flow profiles of the pulmonary veins, making cardiac catheterization and angiocardiography unnecessary in some cases 2.
• For infants and children with TAPVC, a combination of suprasternal, parasternal, subcostal, and apical four-chamber views and their tilting scans can be employed for diagnosis and to trace the course of the anomalous pulmonary venous connection 2.
• In cases of infra-cardiac TAPVC, the risk of pulmonary vein obstruction is high, and percutaneous ductus venosus (DV) stent placement can be used to reduce this risk and improve liver function 3.
• Transcatheter closure of PDA is a standard treatment for patients with signs of left ventricular volume overload due to a ductus, and coils and the Amplatzer Duct Occluder are commonly used devices for PDA closure 4.
• Echocardiography can be used to guide transcatheter closure of PDA in extremely low birth weight infants, eliminating the need for aortograms via femoral arterial access and reducing the risk of complications 5.

Surgical Interventions

• Surgical repair of TAPVC is often necessary, and the type of surgery depends on the individual patient's anatomy and physiology 2, 3.
• In some cases, a stepwise treatment approach may be used, combining percutaneous DV stent placement and occlusion after TAPVC repair to ensure survival from initial surgery to bidirectional cavopulmonary shunt (BCPS) procedure and facilitate subsequent treatment 3.
• The surgical findings should be reviewed and correlated with those of the echocardiography to ensure accurate diagnosis and treatment planning 2.

Management of Associated Conditions

• The management of associated conditions such as ASD and incomplete PDA is crucial in the treatment of infra-cardiac TAPVC 6, 4.
• The treatment of these conditions should be individualized based on the patient's specific anatomy and physiology, and may involve surgical or transcatheter interventions 6, 4.