Left heart hypoplasia operated using double pulmonary arterial banding with double arterial duct stenting: A case report

Abstract

Hypoplastic left heart syndrome is a heterogeneous group of congenital cardiac malformations which associates hypoplastic/aplastic left ventricle, mitral and aortic valve, hypoplastic/atresia and severe aortic artery coarctation, and represents a medical-surgical emergency. We present a case of a newborn hospitalised in three clinics (two clinics from Timisoara and one from Vienna), and operated for hypoplastic left heart syndrome, without aortic coarctation, using a mixed technique cardiovascular repair surgery. The initial therapeutic conduct included maintaining the permeability of the arterial canal with prostaglandin E1. At the Vienna General Hospital, at the age of 17 days, bilateral banding of the pulmonary artery was performed and, at the age of 20 days, during the cardiac catheterisation, the Rashkind procedure (balloon atrial septostomy) was performed, with two stents being implanted in the arterial canal. Postoperative complications were postcardiotomy syndrome, pneumonia with Enterococcus faecalis and Stenotrophomonas maltophilia, sepsis with methicillin-resistant Staphylococcus aureus, coagulopathy, mixed anaemia, and metabolic acidosis. The patient died 1 month after the intervention due to cardiorespiratory arrest, bilateral congestive heart failure, left heart hypoplasia with shunt through the arterial canal and pulmonary artery banding, multiorgan failure, and severe secondary haemorrhagic disease. In conclusion, the initial cardiac surgical reconstruction consisted of a mixed technique, and anticoagulant medical treatment with heparin, antibiotics (bacterial endocarditis prophylaxis to be performed throughout life); postintervention hypoxic and infectious complications resulted in multiorgan failure and death.

A novel KLF13 mutation underlying congenital patent ductus arteriosus and ventricular septal defect, as well as bicuspid aortic valve

“…When minor cardiac structural anomalies are included, such as aneurysm of the atrial septum and bicuspid aortic valve (BAV), which is the most prevalent congenital cardiovascular anomaly with an incidence of 1-2% in the population, the overall prevalence of CHD may be as high as ~5% (2). Based on the occurrence of cardiac lesions in certain locations, CHD is clinically classified into >20 distinct subtypes, including ventricular septal defect (VSD), patent ductus arteriosus (PDA), transposition of the great arteries (TGA), double outlet of the right ventricle (DORV), tricuspid valve atresia (TVA), atrial septal defect (ASD), endocardial cushion defect, aortic stenosis, a right aortic arch, a single ventricle, tetralogy of Fallot, hypoplastic left heart and hypoplastic right heart (3)(4)(5)(6). Irrespective of minor CHD that may resolve spontaneously (3), major CHD may contribute to diminished health-associated quality of life (7)(8)(9), decreased exercise performance (10)(11)(12)(13)(14), delayed neurodevelopment and brain injury (15)(16)(17)(18), ischemic or hemorrhagic cerebral stroke (19)(20)(21), pulmonary arterial hypertension or Eisenmenger syndrome (22)(23)(24), viral pneumonia (25)(26)(27), infective endocarditis (28)(29)(30), acute myocardial infarction (31,32), chronic congestive heart failure (33)(34)(35), ventricular or supraventricular A novel KLF13 mutation underlying congenital patent ductus arteriosus and ventricular septal defect, as well as bicuspid aortic valve arrhythmia…”