Severe dysplasia of the tricuspid valve (unguarded tricuspid anulus): Clinical presentation and surgical treatment

Shukla

et al. 2016

Indian Heart Journal

Congenitally unguarded tricuspid valve (TV) orifice, a variant of TV dysplasia, is a rare malformation with protean manifestations. This report describes a symptomatic adult male with gross right heart failure and atrial fibrillation, who was found to have an unguarded TV orifice with isolation of the trabecular apical cavity of the right ventricle (RV) and muscular ridges separating outflow tract (forme-fruste of the double-chambered RV). The right ventricular outflow tract remained patent.

Section: Discussionmentioning

confidence: 99%

“…Unguarded tricuspid valve (TV) with well-developed right ventricle (RV) and the patent outflow tract constitute a rare congenital anomaly with a few antemortem case reports 1, 2, 3, 4, 5, 6, 7, 8, 9, 10, 11. It is a variety of TV dysplasia, in which there is partial or complete agenesis of the TV tissue 12 .…”

Section: Introductionmentioning

confidence: 99%

Congenitally unguarded tricuspid valve orifice with right ventricular apical isolation in an adult

Shukla

et al. 2016

Indian Heart Journal

“…10,11 A two-ventricle repair approach is often not possible due to the free tricuspid regurgitation and RV dysfunction. 11,12…”

Section: Discussionmentioning

confidence: 99%

“…Both patients had an initial palliation with a systemic‐to‐pulmonary‐artery shunt followed by a bidirectional Glenn shunt 10,11 . A two‐ventricle repair approach is often not possible due to the free tricuspid regurgitation and RV dysfunction 11,12 …”

Section: Discussionmentioning

confidence: 99%

An Unusual Case of Congenitally Unguarded Tricuspid Valve Orifice Associated with Pulmonary Valve Atresia and Intact Ventricular Septum

Takawira¹,

Mitchell²,

Plessis

2010

Echocardiography

We report on a premature neonate who presented with cyanosis soon after birth. She was found on echocardiography to have an unguarded tricuspid valve orifice associated with pulmonary valve atresia and an intact interventricular septum. Owing to the small-sized pulmonary arteries, she was initially managed conservatively with prostaglandin infusion, and at one year of age underwent a successful bidirectional cavo-pulmonary (Glenn) shunt operation. (Echocardiography 2010;27:202-204) Key words: unguarded, tricuspid valve, pulmonary valve atresia, Ebstein's, cavo-pulmonary, FontanThe unguarded tricuspid valve (TV) orifice is a rare congenital heart malformation. It consists of partial or complete agenesis of the TV tissue and its apparatus, and is often associated with pulmonary valve atresia. It has, however, been reported with a patent pulmonary valve (PV). There are little data available on the management of these patients. The literature is limited to case reports that are based mostly on postmortem findings. Herein, we describe the clinical and echocardiographic findings in an infant medically managed until a bidirectional Glenn shunt could be performed. Case Report:The infant is a 1.9 kg, 36 wk gestation female born to a 27-year-old HIV-positive female. The baby was cyanosed at birth and did not respond to oxygen or intubation with mechanical ventilation. On physical examination, the baby was not dysmorphic and the oxygen saturation was 75%. The apex beat was not displaced. A 2/6 ejection systolic murmur was audible in the second left intercostal space. Chest radiography demonstrated mild cardiomegaly with oligemic lung fields. Electrocardiography revealed an HR of 150/min, sinus rhythm, and a QRS axis of +75 degrees. The P-waves were tall and peaked, indicating right atrial enlargement. There were poor right ventricular forces with dominance of the left ventricle.Transthoracic echocardiography (Fig. 1A) demonstrated a large unguarded TV orifice in the normal position. There were no TV leaflets, chordae tendinae, or papillary muscles present. The right atrium (RA) was dilated with the interatrial septum deviating to the left. A small atrial septal defect (ASD) with right-toleft shunting was evident. Color flow Doppler echocardiography demonstrated swirling of blood in the RA, right ventricle (RV), and across the unguarded TV orifice (Fig. 1B). Spectral Doppler interrogation across the unguarded TV orifice demonstrated low velocity systolic-diastolic flow. There was also PV atresia with no prograde flow from the RV across the PV. The pulmonary arteries (PAs) were small and measured only 2 mm. They were fed retrogradely from a patent ductus arteriosus (PDA). Treatment with prostaglandin VR infusion was commenced soon after birth to maintain ductal patency. Her saturations improved from 75% to 90%. The PAs were deemed too small to accommodate a systemic-to-pulmonary-artery shunt. She was therefore maintained on the prostaglandin infusion for two weeks. After it was discontinued, the PDA

“…Those patients can tolerate tricuspid regurgitation well and they become symptomatic only if significant right ventricular dysfunction occurs with or without atrial fibrillation . Surgical treatment is a difficult option due to variable natural history, and surgical results are not too encouraging . Some reports have described the success of the Fontan operation or the total cavo‐pulmonary anastomosis in such cases .…”

Section: Case Reportmentioning

confidence: 99%

A case of unguarded tricuspid valve orifice diagnosed accidentally in an adult

et al. 2019

Unguarded tricuspid orifice is the most extreme of tricuspid valve dysplasia with a very variable natural history. They can tolerate tricuspid regurgitation well, and they become symptomatic only if significant right ventricular dysfunction or atrial fibrillation occurs. Patients with a mild degree of right ventricular dysfunction can survive to adulthood and even reach old age. Surgical treatment is a difficult option due to variable natural history, and surgical results are not too encouraging.