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At least 19 recordsLinked to original sources
PubMed1998

[Prenatal diagnosis of tricuspid atresia].

Tricuspid atresia is the third most common cyanotic cardiac malformation, seen in 1 per cent of children with congenital heart disease. It occurs in 4-5 per cent of congenital heart defects diagnosed prenatally, this difference is a consequence of a high percentage of intrauterine death these severely ill fetuses. Initial surgical treatment is palliation, usually with aortopulmonary shunt. Definitive treatment is the Fontan operation, when the systemic venous return is connected directly to the pulmonary tree. Five-year survival for liveborn patients is 50, but for prenatally diagnosed fetuses only 20%. In Hungary there is only a few survivor. Authors have diagnosed 20 tricuspid atresias during a five-year period by fetal echocardiography. In two cases the postnatal findings were: normal tricuspid valves, univentricular atrio-ventricular connection and stenosis of the pulmonary artery, in one case dysplastic tricuspid valves, hypoplastic right ventricle and stenosis of the pulmonary artery. Authors summarise their experiences with prenatal echocardiography of tricuspid atresia and describe characteristic signs could be found by routine ultrasound screening of this severe cardiac malformation.

Echocardiography↗
PubMed1993

Pregnancy after surgical correction of tricuspid atresia.

Tricuspid atresia comprises 1-3% of congenital heart diseases and it is still uncommon in adult patients. We present a case of tricuspid atresia in a young woman who experienced a successful uncomplicated pregnancy after surgical correction of her disease.

Adult↗
PubMed2005

ZFPM2/FOG2 and HEY2 genes analysis in nonsyndromic tricuspid atresia.

Tricuspid atresia (TriAt), the third most common cyanotic congenital heart defect (CHD), consists of complete lack of tricuspid valve formation, with no connection between the right atrium and the right ventricle. To date, the genetic mechanism responsible of TriAt is still obscure. However, animal models have suggested a role of cardiogenic Zfpm2/Fog2 and Hey2 genes in the pathogenesis of TriAt. Therefore, we screened 40 individuals affected by nonsyndromic TriAt for ZFPM2/FOG2 and HEY2 gene mutations. No pathogenetic mutation has been identified, thus failing to demonstrate a major role of ZFPM2/FOG2 and HEY2 genes in the pathogenesis of human TriAt.

Adolescent↗
PubMed1989

[A seven-year course after Fontan operation in an older adult with tricuspid atresia].

Tricuspid atresia (TA) is a uncommon cardiac anomaly. Most patients with TA will develop severe hypoxia due to insufficient pulmonary flow at an early age and long-term survival without any surgical intervention may not be expected. A 38-year-old male with TA (type Ib) was referred with the complaints of increasing cyanosis and easy fatigability. After diagnosis was confirmed through the cardiac catheterization, modified Fontan operation was performed. In the procedure atrial septal defect was repaired by a patch, stenotic pulmonary valve was closed and prosthetic graft was inserted between the right atrium and the main pulmonary arterial trunk. In literatures about adult with TA, left ventricular dysfunction was generally reported. However, in our case postoperative course was uneventful and his symptoms improved dramatically. After the discharge from hospital, he has done well. But in the cardiac catheterization performed 7 years after operation, the cardiac index was slightly decreased and a significant stenosis along the left anterior descending coronary artery was incidentally discovered. In each laboratory examination after operation slight polycythemia was disclosed.

Cardiac Catheterization↗
PubMed1980

Protein-losing enteropathy after Fontan operation for tricuspid atresia (imperforate tricuspid valve).

Protein-losing enteropathy occurred in a 7-year-old girl with tricuspid atresia, concordant ventriculo-arterial connexions and a relatively large hypoplastic right ventricle, one year after an atrio-ventricular type of Fontan operation by means of a valveless woven Dacron conduit. Severe conduit regurgitation and a marked enlargement of the hypoplastic right ventricle were demonstrated at recatheterization. Insertion of a bioprosthetic valve at the base of the right atrial appendage led to a dramatic clinical recovery of the patient. The use of a valved conduit is recommended when an atrioventricular type of Fontan repair is planned in patients with tricuspid atresia, concordant ventriculo-arterial connexions and relatively large hypoplastic right ventricle.

Aortic Valve Insufficiency↗
PubMedDate not supplied

Absent pulmonary valve with tricuspid atresia or severe tricuspid stenosis: report of three cases and review of the literature.

Absence of the pulmonary valve occurs usually in association with tetralogy of Fallot and occasionally with an atrial septal defect or as an isolated lesion. Very rarely it occurs with tricuspid atresia, intact ventricular septum, and dysplasia of the right ventricular free wall and of the ventricular septum. We present the clinical, anatomic, and histologic findings of a new case, and for the first time, the data from two patients with absent pulmonary valve and severe tricuspid stenosis, who exhibited similar histologic findings. We also reviewed the clinical and anatomic data of 24 previously published cases and compared them with the new cases. In all three new cases, the myocardium of the right ventricle was very abnormal. In the two cases with tricuspid stenosis, large segments of myocardium were replaced with sinusoids and fibrous tissue. In the case with tricuspid atresia, the right ventricular free wall contained only fibroelastic tissue. The ventricular septum in all three patients showed asymmetric hypertrophy and in two of the three patients, multiple sinusoids had replaced large segments of myocardial cells. The left ventricular free wall myocardium and the walls of the great arteries were unremarkable. Our data indicate that myocardial depletion involving the right ventricular free wall and the ventricular septum and its replacement by sinusoids and fibroelastic tissue occur not only in cases of absent pulmonary valve with tricuspid atresia but also in cases of absent pulmonary valve with tricuspid stenosis. The degree of myocardial depletion varies and is more severe when the tricuspid valve is atretic.

Fatal Outcome↗
PubMed1983

Left to right atrial shunting in tricuspid atresia.

In tricuspid atresia, an obligatory right to left shunt occurs at the atrial level. We have observed several patients with left to right interatrial shunts. Data from cardiac catheterisation in 40 consecutive patients were reviewed to determine the frequency and mechanism of left to right shunting in tricuspid atresia. An increase of 6% or more in oxygen saturation between the superior vena cava and the right atrium in two or more sets of saturations, representing a left to right shunt, was present in 29 out of 50 (58%) catheterisations in which the data were adequate. In most, the shunt was also seen cineangiographically in the laevophase. In only two catheterisations was an anatomical cause (ostium primum atrial septal defect in one and anomalous pulmonary venous return in the other) found. In the remaining 27 catheterisations, no anatomical cause was found. Age, Qp:Qs, and mean atrial pressure difference were similar between the shunt and non-shunt groups. In the shunt group right atrial "a" waves were equal to or higher than left atrial "a" waves and left atrial "v" waves were equal to or higher than right atrial "v" waves. Simultaneous pressure recordings (in one patient with left to right atrial shunt) from the left atrium and right atrium with isosensitised miniature pressure transducers mounted 5 cm apart showed (1) a higher pressure in the right atrium than in the left atrium during atrial systole and (2) a higher pressure in the left atrium than in the right atrium during atrial disatole. It is concluded that (a) left to right shunt across the atrial septum occurs frequently in tricuspid atresia and (b) the left to right shunt is the result of instantaneous pressure differences between the atria.

Adolescent↗
PubMed1991

Palliative repair of aortic atresia associated with tricuspid atresia and transposition of the great arteries.

Successful palliative repair of aortic atresia and hypoplastic aortic arch associated with tricuspid atresia in a neonate is described. The repair consisted of reconstruction of the hypoplastic aortic arch with an equine pericardial patch, division of the patient ductus arteriosus, connection of the pulmonary artery to the aorta, implantation of the proximal part of the ascending aorta into the main pulmonary artery, and anastomosis of a polytetrafluoroethylene graft 5 mm in diameter between the right ventricular outflow tract and the central pulmonary artery, which was transferred anteriorly to the main pulmonary artery.

Abnormalities, Multiple↗
PubMed1984

Tricuspid atresia with overriding imperforate tricuspid membrane: an anatomic variant.

An anatomical study of 34 specimens presenting with tricuspid atresia, in which particular emphasis was devoted to the atrioventricular junctional area, revealed 5 cases with an imperforate valve. In 1 heart the valve was completely committed to the right ventricle. In the other 4, however, the membrane was related not only to the right ventricle but also to the left ventricle. A hypoplastic right ventricular inlet portion was found in these 4 specimens. In 2 cases the tricuspid membrane overrode an inconspicuous inlet septal defect. In the other 2 hearts, as studied macroscopically, no defect was present underneath the membrane, which was connected to both right and left ventricles. These 4 specimens also had other ventricular septal defects. In classical tricuspid atresia, characterized by absence of the inlet portion of the right ventricle, the inlet septum is absent. In contrast, these 4 hearts did have an hypoplastic inlet septum.

Atrioventricular Node↗
PubMed1989

[The Fontan operation in tricuspid atresia. Effect of age, shunt and type of atrial pulmonary connection in tricuspid atresia].

Between 1977 and 1988, 20 patients underwent the Fontan operation. Their age ranged from 4 to 24 years (mean 11 years). The basic disease was tricuspid atresia with ventriculo arterial concordance and right ventricular hypoplasia. Pulmonary stenosis was present in 16 patients. Twenty-eight palliative procedures had been performed previously in the group of patients presenting some forms of pulmonary stenosis. In 2 cases a non-valved conduit has been used for correction, in a third case a valve conduit was utilised, and in a fourth case a right atrioventricular connection was carried out. In the remaining 16 patients, a wide posterior connection between the right atrium and the pulmonary arteries was performed. There were 2 early deaths (10%), and 2 late deaths (10%). After a follow-up ranging from 6 months to 11 years, all survivors are in a satisfying functional condition. We think that a wide unrestricting atriopulmonary anastomosis is mainly responsible for those good functional results. Age at operation has not influenced the results. Previous and long standing shunts may be responsible for immediate and late postoperate cardiac failure. Consequently, we now prefer an earlier corrective procedure, particularly when the patient has a patent systemic pulmonary shunt.

Adolescent↗
PubMed1977

Tricuspid atresia and pregnancy.

Tricuspid atresia is one of the rarest heart defects, and it is rarely associated with prolonged survival. This paper describes the third known case of pregnancy associated with tricuspid atresia. This is the first detailed report of the clinical course of a patient with this disorder. Complications included heart failure, multiple pulmonary emboli, cerebral embolization, and premature labor. Recommendations are made regarding patient care.

Adult↗
PubMed1997

Tricuspid atresia and 22q11 deletion.

Tricuspid atresia has not been reported in 22q11 microdeletions causing DiGeorge and velo-cardio-facial syndromes. We investigated the prevalence of 22q11 hemizygosity in 26 children with tricuspid atresia. Fluorescent hybridization with the Sc11.1 probe demonstrated a 22q11 microdeletion in 2 patients, one with and another without transposition of the great arteries. Both deletion patients had minor facial anomalies characteristic of DiGeorge syndrome. The present observations suggest that tricuspid atresia should be included in the list of cardiac malformations seen in del22q11 syndromes.

Adult↗
PubMed1989

Course of tricuspid atresia in the Fontan era.

Tricuspid atresia is an uncommon form of congenital heart disease and long-term survival was rare before the Fontan era. It was thought that the long-term survival of patients with tricuspid atresia would be improved by the introduction of the Fontan procedure and its subsequent modifications. This study reviews the clinical course of 84 patients with tricuspid atresia identified in the first year of life in the Fontan era. Prior palliative operations, their results and their ultimate application for the Fontan procedure were considered. Eleven patients died before surgical intervention and 5 did not undergo catheterization or echocardiographic confirmation before death. Five children underwent the Fontan procedure without prior palliation and 1 child does not require palliation at the present time. Sixty-seven patients (80%) had surgical procedures before evaluation for the suitability of a Fontan operation. Thirty-four patients had a second surgical palliation and 9 patients had a third palliation. The surgical mortalities for the first, second and third palliative surgery were 17.9, 17.6 and 0%, respectively. Thirty-two patients (38%) underwent the Fontan procedure and 2 deaths occurred (6%). An estimate of the probability of surviving for 1 year was 64% (95% confidence limits 54 to 74%) and that of 8 years was 55% (95% confidence limits 44 to 66%).

Blood Vessel Prosthesis↗