Subpulmonic Obstruction by Aneurysmal Tricuspid Valve Tissue in a Perimembranous Ventricular Septal Defect

Abstract

A 10-year-old female patient presented with dyspnea during play with peers. She had been diagnosed with a large perimembranous ventricular septal defect neonatally but had since been lost to follow-up. On physical examination, she was fully saturated, with a 4/6 systolic murmur along the left sternal border. Transthoracic echocardiography identified a restrictive perimembranous ventricular septal defect but with bidirectional flow and peak gradient of 110 mm Hg out the right ventricular outflow tract. Catheterization revealed suprasystemic pressures in the right ventricular apex with a peak gradient of 107 mm Hg between the right ventricle and the pulmonary artery. On angiography (A, Online Video 1), aneurysmal septal tissue (black arrow) could be seen oscillating within the right ventricular outflow tract in addition to mild infundibular hypertrophy. Pre-operative transesophageal echocardiography (B, white arrow denoting aneurysmal tissue, black arrow denoting pulmonary valve; C, black arrow denoting aneurysmal tissue, white arrow denoting perimembranous septal defect; Online Videos 2 and 3) and ultimately surgical resection (D, black arrow denoting aneurysmal tricuspid tissue) confirmed the diagnosis of subpulmonic obstruction by aneurysmal tricuspid valve tissue and infundibular hypertrophy.

Discrete subpulmonic membrane in association with isolated severe pulmonary valvar stenosis

“…Other causes of subpulmonic obstruction reported in the literature include aneurysm of the membranous septum [5,6], aneurysmal tricuspid valve tissue in the setting of membranous ventricular septal defect [7], double-chambered right ventricle [8], and unruptured sinus of Valsalva aneurysm [9,10]. A single case of right ventricular outflow tract obstruction resulting from a tuberculoma in a patient with ventricular septal defect and aneurysm of the membranous septum has also been reported [11].…”

Right Ventricular Outflow Tract Obstruction by an Aneurysm of the Ventricular Membranous Septum: A Systematic Review of Case Reports

“…We included only case reports and case series with fulltext publications. Cases with a right ventricle-pulmonary artery systolic pressure gradient of ≥35 mm Hg were selected, including case reports 6,7 published as "Image in Cardiology." Characteristics of each patient were collected on a Microsoft Excel sheet and analyzed for several items.…”

“…We included only case reports and case series with full-text publications. Cases with a right ventricle-pulmonary artery systolic pressure gradient of ≥35 mm Hg were selected, including case reports 6,7 published as “Image in Cardiology.” Characteristics of each patient were collected on a Microsoft Excel sheet and analyzed for several items. Data elements collected during the extraction process included the following: author, year of publication, patients’ characteristics including age at surgery, sex, thesis title showing the aneurysm of the ventricular membranous septum, the morphology of an aneurysm diagnosed by left ventriculography, echocardiography or surgery, the size of both an aneurysm (tissue tag) and a VSD measured by echocardiography or surgery, and a right ventricle-pulmonary artery systolic pressure gradient measured by Doppler echo and/or cardiac catheterization, the interventricular shunt, associated heart anomaly, chromosomal anomaly, operation, and tricuspid valve findings.…”

“…20 One of 19 patients above the age of 18 had reported having high blood pressure. 35 Childhood symptoms included dyspnea during play in a 10-year-old girl, 7 and palpitations during exercise in an 11-year-old boy. 8 A four-year-old girl suffered convulsions due to a cerebral embolism during subacute bacterial endocarditis.…”

“…The pouch formation of the septal leaflet of the tricuspid valve in a perimembranous VSD causes a diminution of the shunt The right border of the AN sac was continuous with the TV Flege et al 13 Excision Direct AN of the STL Chesler et al 14 Resection Closure TP (ATL) Iida et al 8 Resection Mattress suture ND Cosio et al 15 Resection Patch (Felt) Membranous sac attached to the TV Silvestre et al 16 Plication ND TV competent Gomes et al 17 Excision Closure Wind-sock-flap (in relation to STL) Bonvicini et al 18 Reinforce Patch (Teflon) ND Nagaoka et al 9 Excision Patch (ND) ND Johnson et al 19 Excision Closed primarily ND Chadha et al 20 Excision Patch (Dacron) ND Sharma et al 21 Excision (major portion) Obliterated The membranous sac involved the base of the TV Kaneko et al 22 Closure 24 Excision Patch (Dacron) ND Güneşet al 25 Surgery (−) Love et al 6 Surgery (−) Sugino et al 26 Resection Direct ND Chen et al 27 Resection Small VSD closure Dilated Eason et al 7 Resection ND Aneurysmal TV tissue Altunbas et al 28 Resection Internal patch closure ND Bamous et al 29 Excision Patch (Dacron) ND Mahesh et al 30 Resection Patch (Dacron) AN formed by STL Privitera et al 31 Surgery (−) Velicki et al 32 Plication (transaortic) ND ND Bakr et al 33 Resection Patch (Dacron) ND Bride et al 34 Resection Patch (Gore-Tex) AN originate from STL Yamashita et al 10 Resection(−) Patch (Gore-Tex) AN continuing from STL Herron et al 35 Surgery (−) volume and can lead to spontaneous closure. 3 After pouch formation, it is extremely rare to require surgery for the right ventricular outflow tract obstruction.…”