Successful balloon valvuloplasty of a subpulmonic membrane associated with cor triatriatum dexter: a case report

Abstract

Background Subpulmonic membrane as a cause of right ventricular outflow tract obstruction in patients with concordant ventriculoarterial connection and intact ventricular septum is considered to be rare. Association with cor triatriatum dexter and success of subpulmonic balloon valvuloplasty have never been reported, at least to the best of our knowledge. Case presentation A 3-year-old Moroccan boy was referred to our tertiary care hospital with complaints of dyspnea on moderate exertion. A physical examination revealed parasternal lift, systolic thrill, and a 4/6 ejection systolic murmur, best heard over the left second intercostal space. His oxygen saturation was 99% on room air. Two-dimensional echocardiography showed a discrete circumferential membrane just below the pulmonic valve and a right atrial membrane. Continuous wave Doppler interrogation showed peak systolic pressure gradient of 85 mmHg across the subpulmonic membrane and no significant gradient across the right atrial membrane. Balloon dilation of the subpulmonic membrane was performed and the pressure gradient came down to 50 mmHg. During follow-up, he reported marked improvement in terms of exercise tolerance. Transthoracic echocardiography showed residual pressure gradient of approximately 40 mmHg across the membrane. Surgery resection of the two membranes was programmed, but he died after an extracardiac disease (appendicular peritonitis). Conclusions Subpulmonic membrane as an isolated cause of right ventricular outflow tract obstruction is rare. Its association with cor triatriatum dexter is even less common. The result of percutaneous balloon valvuloplasty of subpulmonic membrane is an interesting alternative while waiting for surgery. Surgery is currently the preferred modality of treatment with the resection of both right atrial and subpulmonic membranes.

Surgical management of divided atrial chambers

“…Surgical resection of the venous valves under cardiopulmonary bypass, particularly if forming a spinnaker‐like malformation, is the treatment of choice for symptomatic patients with significant right ventricular inflow obstruction, cyanosis, complex anatomy, and associated congenital heart diseases 27 . There are isolated reports of successful transcatheter balloon dilation, but this can only be employed in patients with less obstructive lesions with uncomplicated anatomy 68–70 . Percutaneous catheter‐based disruption of the venous valves have been reported when identified as forming a Chiari network, although the redundant network makes catheterization difficult because of difficult navigation or entrapment of the sheaths and/or the closure of an associated interatrial communication 68–73,87,88 …”

“…On this basis, it proved possible to make an individualized review of 193 investigations 1–193 . These findings were then incorporated, as far as possible, with the results of a systematic review of ...…”

“…Division of the morphologically left atrium has been estimated to be around 0.1% to 0.4%, while the persistence of the valves of the systemic venous sinus accounts for 0.025%–0.1% of congenital heart diseases 22,29–31 . Associated congenital and acquired cardiac lesions have been reported in one‐third to three‐quarters of those with divided left atriums and in one‐half of those in whom the morphologically right atrium is divided 23–28,31–91 . Limited information is available, however, on the short‐ and long‐term outcomes of treatment for these rare conditions.…”

Cor triatriatum dexter in children: Literature review and case report

“…(5) The presence of associated right ventricular outflow tract obstruction at different levels. 2 , 12 , 17 (6) Predominant obstruction of inferior vena cava flow by the RA membrane with no associated ASD. 6 , 7 …”

Cor Triatriatum Dexter: A Case Report in a 70-Year-Old Male

“…In the past, the mainstay of treatment for symptomatic patients was surgical resection of the dividing membrane [ 7 ]. Recently, however, percutaneous catheter disruption of the membrane has been suggested as a preferred alternative to open heart surgery [ 10 , 11 ].…”