Hypoplastic right heart syndrome (HRHS) is a rare cyanotic heart disease due to the underdevelopment of right heart structures (Tricuspid valve, RV, Pulmonary valve and pulmonary artery). The spectrum of disorders varies from simple hypoplastic RV with or without pulmonary stenosis to grossly underdeveloped RV with pulmonary and/or tricuspid atresia [1, 2]. Ventricular size can vary from grossly hypoplastic to normal-sized chambers [3]. HRHS is usually associated with ASD (atrial septal defect), VSD (ventricular septal defect), AVCD (atrioventricular cushion defect) and other complex defects. Clinical presentation depends on the extent of development of RV, pulmonary circulation, and usually present early in the life. The goals of early palliation include relief of cyanosis and duct dependence by establishing adequate pulmonary flow for development of right-sided structures. Treatment options include surgical systemic pulmonary shunt and percutaneous techniques like PDA stenting or pulmonary valve perforation. The difficulty in ductal stenting stems from the fact that ducts in these patients are tortuous and so incomplete stenting, stent migration can occur. Surgical correction at neonatal stage has higher mortality [4]. In the subset of patients with short-segment atresia management, options are well studied. Transcatheter pulmonary valve perforation offers a physiological approach for pulmonary flow and development of pulmonary arteries for future palliative surgeries. It also helps in the growth of the RV by the induced pulmonic regurgitation to a near normal size [3]. Also, the authors have experience of PV perforation and stenting of the RVOT in a sick infant (unpublished work); we felt that PV perforation offers a better palliative procedure short of surgery. The advantage with pulmonary valve perforation is that the pulmonary flow is more physiological and the induced pulmonary regurgitation helps in the growth of the right ventricle for future biventricular repair. In a series of 13 patients who underwent RVOT perforation, there was significant growth of the TV annulus and reduction in TR during at a mean follow-up of 13.2 months [5]. On the contrary there have been studies where there was no significant growth of the right ventricle following perforation [6,7,8]. The surgical aorto pulmonary shunting is a well-established palliative surgery in HRHS, but carries an increased risk in under-weight neonates. Complication with surgical shunting include pulmonary over-circulation, stenosis at the anastomosis site, asymmetric development of pulmonary vasculature, distortion of the vessels, nerve injury, and chylothorax [9]. In these patient’s assessment of the atretic pulmonary valve for its thickness, diameter of the RVOT, MPA, and branch PA’s is essential for selecting suitable patients. Perforation using hydrophilic coronary wires or radiofrequency ablation has been described in the literature [10,11,12]. Surgical correction (pulmonary valvotomy) at neonatal prove to be detrimental in some series [13]. Humpl et al. in their series of 50 patients of PA with IVS, attempted PV perforation in 30 patients by either stiff end of the coronary guide wire or RF ablation with a procedural success in 27 patients. They could find that with PV perforation though RV did not increase according to body growth at early follow up, it is adequate to maintain a biventricular circulation [10]. In a series of 33 patients of PA with IVS by Alwi et al. where RF ablation with balloon dilatation was compared with surgical valvotomy and BT shunt, percutaneous procedure was more efficacious and safe [1]. Procedural success has been described as establishment of adequate pulmonary flow, weaning from prostaglandin, and without requiring reintervention [8]. Complications are rare including perforation with tamponade, arrhythmia, and vascular injury [10]. Surgical correction (pulmonary valvotomy) at early stage prove to be detrimental in some series [13]. In our case, we proceeded with percutaneous intervention, as less invasive procedure in a sick neonate is probably safer. However, the child succumbed to pneumonia-sepsis after 5 weeks of procedure.
