A 12-year-old girl with hypoplastic right heart, double inlet left ventricle, and transposition of the great arteries had a lateral tunnel Fontan operation 8 years previously. She developed symptoms related to a combination of stenosis in the inferior caval venous pathway, a fenestration, and leak in the lateral tunnel. These were successfully treated with a custom-made stent graft.

Arterial switch surgery for d-transposition of great arteries is usually performed in the first 2 weeks of life before the left ventricle regresses. The arterial switch surgery that helps achieve anatomic and physiologic correction of this condition has better long-term outcomes than other surgical approaches. The procedure may still be attempted at an older age where the left ventricle has not regressed. We report a rare case of a 12-year-old child with d-transposition of great arteries, a remote muscular ventricular septal defect and isolated valvar pulmonic stenosis, which was corrected by an arterial switch surgery.

We report an infantile case of Loeys–Dietz syndrome prenatally diagnosed with congenital complex heart disease – double outlet right ventricle and interruption of the aortic arch. The patient also showed prominent dilatation of the main pulmonary artery. Emergency bilateral pulmonary artery banding was performed on the 9th day. However, on the 21st day, the patient died of massive bleeding due to rupture of the right pulmonary artery. Subsequently, a mutation of the TGFBR1 gene was detected. As cardiovascular lesions of Loeys–Dietz syndrome appear early and progress rapidly, the prognosis is generally poor. Patients require periodic examination and early intervention with medical therapy such as Losartan administration and surgical therapy. Early genetic screening is thought to be useful for the prediction of complications as well as vascular disease.