Congenitally unguarded tricuspid valve orifice with a giant right atrium and a massive clot in an asymptomatic adult.

Congenitally unguarded tricuspid valve orifice, a variant of tricuspid valve dysplasia, is a rare malformation with protean manifestations. This report describes an asymptomatic adult who, on echocardiographic examination ordered in view of an abnormal 12-lead surface electrocardiogram and plain chest X-ray, was found to have an unguarded tricuspid valve orifice with a giant right atrium (12 x 10 cm), intense spontaneous echo contrast and a large right atrial clot.     

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

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.     

Echocardiographic spectrum of congenitally unguarded tricuspid valve orifice and patent right ventricular outflow tract

A series of nine consecutive patients with unguarded tricuspid valve orifice as a result of partial or complete agenesis of the valvar tissue and patent right ventricular outflow tract is reported. Clinical manifestations were cyanosis, severe right ventricular failure and incidental echocardiographic detection in a young patient with dilated cardiomyopathy. This series contains the oldest reported patient with this malady, who was misdiagnosed as portal hypertension for 10 years. This study, with possibly the largest number of patients reported so far, sheds some light on the natural history of a rare entity.     

Hepatocellular carcinoma with right atrial extension in a young patient with congenitally unguarded tricuspid orifice

We report a case of a young female patient with congenitally unguarded tricuspid orifice and underdeveloped pulmonary valve leaflets, who had developed severe right-sided congestive heart failure and cardiac cirrhosis early in life. She was admitted to our hospital with deterioration of dyspnea and abdominal tenderness. Hepatocellular carcinoma was diagnosed on the basis of computed tomography findings, high plasma alpha-fetoprotein levels and evidence of chronic hepatitis B viral infection. Transthoracic echocardiogram revealed a mass into the right atrium, considered to be an extension of the tumor.     

Echocardiographic evaluation of normal and prolapsed tricuspid valve leaflets

The tricuspid valve was examined by 2-dimensional (2-D) echocardiography in 14 patients with tricuspid valve prolapse (TVP) and in 16 normal subjects. Individual leaflets were identified anatomically and for frequency of prolapse. Maximal and minimal anular sizes were measured. Multiple tomograms of the tricuspid anulus were recorded at 30 degrees intervals around the tricuspid anulus with the transducer placed at the right ventricular apex. Anuli were reconstructed from the 6 planes and corrected for body surface area. Three leaflets of the tricuspid valve could be anatomically identified in all patients. Prolapse of all 3 leaflets was observed in 6 patients, 2 leaflets in 5 and 1 in 3. Frequency of individual leaflet prolapse was 93% for the septal cusp, 86% for the anterior and 43% for the posterior. Maximal anular circumference and area in TVP were 7.9 +/- 0.6 and 8.9 +/- 1.3 cm2/m2, respectively--significantly larger than values in normal subjects (6.4 +/- 0.5 cm/m2 and 6.1 +/- 0.9 cm2/m2, respectively) (p less than 0.001). Percent reductions in circumference and area in TVP were 14 +/- 3 and 25 +/- 5%, respectively--significantly smaller values than in normal subjects (19 +/- 4 and 33 +/- 4%, respectively). Tricuspid regurgitation (TR) was detected by contrast echocardiography in 7 of 14 patients with TVP. The severity of TR appeared to be minimal in 6 of the 7 patients, and was not associated with an increase in anular size. Thus, TVP is associated with anular dilatation irrespective of associated TR, probably as a primary pathologic characteristic.     

A case of congenital tricuspid valve abnormality showing six leaflets

A-59-year old woman died from heart failure associated withtricuspid regurgitation and pulmonary hypertension.Necropsyrevealed that her tricuspid valve was composed of six leaflets.We found no previous report of this type of tricuspid valveabnormality.     

Prolapse of atrioventricular valve leaflets in the setting of double orifice

Prolapse of the valve leaflets is reported in 2 cases in the setting of double orifice atrioventricular valve. Diagnosis in the first case, an asymptomatic 8-year-old female, was achieved by echocardiography and consisted in prolapsing leaflets of a double orifice ("bridge type") mitral valve. The second patient, a 24-year-old male, died suddenly. Postmortem examination revealed prolapsing atrioventricular valves, with double orifice ("holy type") of the septal leaflet of the tricuspid valve. There was disruption of the penetrating atrioventricular bundle into multiple fragments with fasciculo-ventricular by-pass fibers.     

Congenital absence of pulmonary valve leaflets.

Congenital absence of pulmonary valve leaflets is an uncommon condition usually associated with ventricular septal defect and an obstructive pulmonary valve ring. Twenty-one patients with these malformations are described. Twenty had an associated ventricular septal defect with ventriculoarterial concordance, and one also had transposition of the great arteries, ventricular septal defect, and obstructive pulmonary valve ring. The clinical features, cardiac catheterisation findings, and angiocardiographic results are presented. Twelve patients underwent cardiac surgery. Three patients died, one in the early, and the other two in the late postoperative period. The results, according to the surgical technique employed and postoperative cardiac catheterisation findings, showed that patients in whom the bioprostheses were implanted in the pulmonary position had a better late follow-up.     

Papillary fibroblastoma of tricuspid valve with pulmonary embolization

A 53-year-old man was diagnosed with a papillary fibroelastoma of the tricuspid valve with pulmonary embolization and associated coronary artery disease. He underwent excision of the tumor and coronary bypass grafting. Due to potential embolization, it is recommended that all such fibroelastomas be excised.     

Parachute tricuspid valve associated with Fallot's tetralogy]

Parachute deformity of the tricuspid valve was observed with a severe form of Fallot's Tetralogy with pulmonary atresia in a newborn. Signs of tricuspid stenosis (peripheral oedema, right atrial dilatation, hepatomegaly and conduction defects) were associated with the clinical signs of the principal malformation (severe hypoxaemia). To our knowledge, this is the first reported case of parachute deformity of the tricuspid valve.     

Double orifice mitral valve associated with endocardial cushion defect

Duplication of the mitral valve is a rare congenital cardiac anomaly. We encountered a case of duplication of the mitral valve associated with a partial form of endocardial cushion defect in a 6-year-old girl. The mitral orifice was separated by a fibrous tissue and each orifice provided papillary muscle and complete subvalvular mechanisms except for the cleft region. The fibrous tissue also provided a subvalvular apparatus. The cleft was repaired without complication. The short axis view of the two-dimensional echocardiogram demonstrated two separate holes in the mitral valve, which constituted the most obvious diagnostic sign prior to surgery.     

Double orifice tricuspid valve with left atrioventricular valve atresia and univentricular connexion to a dominant left ventricle

We present a case of univentricular connexion to a dominant left ventricle with double orifice tricuspid valve, left atrioventricular valvar atresia, discordant ventriculo-arterial connexion and aortic arch hypoplasia. Diagnosis was made by cross-sectional echocardiography and angiocardiography. This case is exceedingly rare.     

Premature pulmonary valve opening in a patient with absent tricuspid valve.

Echocardiography (M-mode and cross-sectional) disclosed premature opening of the pulmonary valve (very deep "a" wave) in a young man who underwent tricuspid valvulectomy without valve replacement 32 months before for refractory bacterial endocarditis. Cardiac catheterisation showed a prominent atrial wave on the pulmonary artery pressure curve. Thus, part of the right ventricular ejection appeared to occur before the onset of ventricular systole, as a result of powerful right atrial contraction.     

Unmasking pulmonary hypertension following tricuspid valve replacement

In this Viewpoint, we highlight a possible hemodynamic problem arising following tricuspid valve replacement (TVR) in patients with severe chronic tricuspid regurgitation, represented by “unmasking” of pulmonary hypertension (PH) following the surgery. We share an observation that should alert cardiologists to the fact that this increasingly utilized surgery is not risk free, and careful assessment of the right ventricular function and pulmonary circulation preoperatively is extremely important, especially in patients with preexisting risk factors for PH, since TVR may lead to a sudden increase in right ventricular afterload.     

"Balloon valvulotomy" of congenital pulmonary valve stenosis with tricuspid valve insufficiency

The rare congenital anomaly of pulmonary valve stenosis and massive tricuspid valve insufficiency with intact ventricular septum is a lethal condition without reported survival after attempted treatment. In a neonate suffering from this syndrome, the pulmonary valve stenosis was relieved by rupturing the fused valve with a balloon catheter introduced transvenously. The desperate condition of the patient quickly improved after this procedure, with subsequent disappearance of the tricuspid valve incompetence. Balloon rupturing of fused valves at angiography may represent a therapeutic alternative in cases in which surgical valvulotomy is associated with a high mortality.