scholarly journals Fontan operation for tricuspid atresia with absent pulmonary valve: a case series

2021 ◽  
pp. 1-2
Author(s):  
Moyu Hasegawa ◽  
Shigemitsu Iwai ◽  
Yosuke Kugo
Keyword(s):  
Poor Prognosis ◽  
Pulmonary Valve ◽  
Surgical Intervention ◽  
Fontan Operation ◽  
Case Series ◽  
Ventricular Myocardium ◽  
Tricuspid Atresia ◽  
Absent Pulmonary Valve ◽  
The Right ◽  
Congenital Heart Anomaly

Abstract Combined tricuspid atresia and absent pulmonary valve with dysplasia of the right ventricular myocardium is a very rare congenital heart anomaly with a poor prognosis. We present three cases of this rare disease that reached the Fontan operation without prior surgical intervention of the right ventricle or pulmonary artery; no right ventriclar dilation was detected. All patients had uneventful post-operative courses.

Tricuspid atresia with absent pulmonary valve and intact ventricular septum: successful bidirectional cavopulmonary anastomosis with complete exclusion of the right ventricle

2019 ◽  
Vol 30 (1) ◽  
pp. 126-128
Author(s):  
Cheul Lee ◽  
Kyung Min Kim ◽  
Jae Young Lee ◽  
Jihong Yoon
Keyword(s):  
Right Ventricle ◽  
Pulmonary Valve ◽  
Tricuspid Atresia ◽  
Ventricular Septum ◽  
Intact Ventricular Septum ◽  
Absent Pulmonary Valve ◽  
Cavopulmonary Anastomosis ◽  
Complete Exclusion ◽  
Bidirectional Cavopulmonary Anastomosis ◽  
The Right

AbstractTricuspid atresia with absent pulmonary valve and intact ventricular septum is an extremely rare cardiac malformation, historically associated with a poor prognosis. Only a few cases with successful surgical palliation have been reported in the literature. We present the case of an 8-month-old infant with this malformation who underwent successful bidirectional cavopulmonary anastomosis with complete exclusion of the right ventricle.

Absent Pulmonary Valve with Tricuspid Atresia or Severe Tricuspid Stenosis: Report of Three Cases and Review of the Literature

2000 ◽  
Vol 3 (4) ◽  
pp. 353-366 ◽  
Author(s):  
Silvio Litovsky ◽  
Michael Choy ◽  
Jeanny Park ◽  
Mark Parrish ◽  
Brenda Waters ◽  
...  
Keyword(s):  
Right Ventricular ◽  
Pulmonary Valve ◽  
Left Ventricular ◽  
Tricuspid Atresia ◽  
Ventricular Free Wall ◽  
Ventricular Septum ◽  
Free Wall ◽  
Absent Pulmonary Valve ◽  
Right Ventricular Free Wall ◽  
The Right

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.

scholarly journals A Case of Prenatally Diagnosed Uhl’s Anomaly with Absent Pulmonary Valve Leaflets and Dysplastic Tricuspid Valve

Children ◽  
2021 ◽  
Vol 8 (3) ◽  
pp. 190
Author(s):  
Taehong Kim ◽  
Hoon Ko ◽  
Joung-Hee Byun ◽  
Hyoung Doo Lee ◽  
Hyungtae Kim ◽  
...  
Keyword(s):  
Right Ventricular ◽  
Tricuspid Valve ◽  
Pulmonary Valve ◽  
Fetal Echocardiography ◽  
Right Heart Failure ◽  
Ventricular Myocardium ◽  
Fetal Life ◽  
Absent Pulmonary Valve ◽  
Rare Malformation ◽  
The Right

Uhl’s anomaly is a very rare malformation of unknown cause, characterized by complete or partial absence of the right ventricular myocardium. The cardiac malformation causes progressive right heart failure, increased right-sided cardiac pressure, massive peripheral edema, and ascites. Patients usually present in infancy and rarely survive to adulthood. Previously, diagnosis was made at post-mortem evaluation, but advances in cardiac imaging now permit diagnosis during fetal life. We report a case of Uhl’s anomaly in a newborn baby imaged at 23 + 3 weeks of gestation by fetal echocardiography. There was an aneurysmally dilated thin-walled right ventricle with hypertrophy of the right ventricular apical muscles, the tricuspid valve was dysplastic, and the pulmonary valve leaflets were absent.

Surgical management of tricuspid atresia with absent pulmonary valve

2016 ◽  
Vol 42 ◽  
pp. 67
Author(s):  
T.K. Susheel Kumar ◽  
Ronak Naik ◽  
Mohammed Al-Sheikh Ali ◽  
Christopher Knott-Craig
Keyword(s):  
Surgical Management ◽  
Pulmonary Valve ◽  
Tricuspid Atresia ◽  
Absent Pulmonary Valve

Defective regional myocardial development and vascularization in one variant of tricuspid atresia—clinical and necropsy findings in three cases

1992 ◽  
Vol 2 (1) ◽  
pp. 42-52 ◽  
Author(s):  
William N. O'Connor ◽  
Carol M. Cottrill ◽  
Michael T. Marion ◽  
Jacqueline A. Noonan
Keyword(s):  
Coronary Artery ◽  
Right Ventricle ◽  
Tricuspid Valve ◽  
Right Coronary Artery ◽  
Interventricular Septum ◽  
Ventricular Myocardium ◽  
Tricuspid Atresia ◽  
Subaortic Stenosis ◽  
Myocardial Development ◽  
The Right

SummaryFive previously reported cases have established the combination of a small right ventricle (sometimes with Uhl's anomaly), imperforate tricuspid valve with fibrotic tensor apparatus, congenital absence of the pulmonary valve, and an intact interventricular septum with muscular subaortic stenosis as a rare variant of tricuspid atresia. In this study of three new autopsy cases, we additionally identified Ebstein's malformation of the imperforate tricuspid valve, partial Uhl's anomaly, regional dysplasia of right ventricular myocardium and thinning of the interventricular septum by intramyocardial sinusoids from the right ventricle. All three new cases had epicardial anomalies of the right coronary artery—a fistula to right ventricle, ostial stenosis and proximal arterial hypoplasia. Sinusoidal connections from the right ventricle to the right coronary artery and to the sclerotic left anterior descending artery were identified in serial sections of the right ventricle and septum. Myofiber disarray, with thick walled intramyocardial arteries and sinusoids from left ventricle, involved the bulging subaortic interventricular septum. These studies are consistent with the hypothesis that defective development of the the right ventricle, along with its blood supply and associated atrioventricular and arterial valves, may underlie this unusual form of congenital heart disease.

Absent pulmonary valve in tricuspid atresia with left ventricular outflow tract obstruction and patent arterial duct

1997 ◽  
Vol 7 (1) ◽  
pp. 94-97 ◽  
Author(s):  
Robert P. Lemke ◽  
Neils G. Giddins ◽  
Jonah N.K. Odim
Keyword(s):  
Septal Defect ◽  
Pulmonary Valve ◽  
Ventricular Outflow Tract ◽  
Left Ventricular ◽  
Tricuspid Atresia ◽  
Absent Pulmonary Valve ◽  
Arterial Duct ◽  
Left Ventricular Outflow ◽  
Patent Arterial Duct ◽  
Absent Pulmonary Valve Syndrome

AbstractWe describe a neonate with tricuspid atresia, absent pulmonary valve, right ventricular hypoplasia, small venrricular septal defect, left ventricular ourflow tract obstruction and a patent arterial duct. These finding were diagnosed by echocardiography and confirmed by cardiac catherterization and postmortem examination. This is a unique report of a functionally single ventricle variant of absent pulmonary valve syndrome and biventricular outlet obstuction.

Anomalous origin of the right pulmonary artery from the ascending aorta accompanied by absent pulmonary valve syndrome and right-sided aortic arch: a rare case in adult congenital heart disease

2014 ◽  
Vol 25 (4) ◽  
pp. 794-796 ◽  
Author(s):  
Zahra Khajali ◽  
Ali Mohammadzadeh ◽  
Marzieh Khayatzadeh
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Pulmonary Artery ◽  
Congenital Heart ◽  
Pulmonary Valve ◽  
Ascending Aorta ◽  
Absent Pulmonary Valve ◽  
Right Pulmonary Artery ◽  
The Right ◽  
Absent Pulmonary Valve Syndrome

AbstractWe present a rare congenital heart disease in a 20-year-old man with anomalous origin of the right pulmonary artery from the ascending aorta, accompanied by absent pulmonary valve syndrome, and a right-sided aortic arch suspected initially in transthoracic echocardiography and subsequently confirmed by cardiac catheterisation and computed tomography angiography.

Pulmonary Valve Endocarditis: The Potential Utility of Multimodal Imaging Prior to Surgery

2020 ◽  
Vol 11 (2) ◽  
pp. 192-197
Author(s):  
Kristin M. Corey ◽  
Michael Jay Campbell ◽  
Kevin D. Hill ◽  
Christoph P. Hornik ◽  
Richard Krasuski ◽  
...  
Keyword(s):  
Multimodal Imaging ◽  
Pulmonary Valve ◽  
Case Series ◽  
Right Ventricular Outflow Tract ◽  
Ventricular Outflow Tract ◽  
Duke Criteria ◽  
Positron Emission ◽  
Surgical Data ◽  
Major Criterion ◽  
The Right

Background: The presence of echocardiographic (echo) evidence is a major criterion for the diagnosis of infective endocarditis (IE) by modified Duke criteria. Pulmonary valve (PV) IE, however, can be challenging to identify by echo. We sought to evaluate the added utility of multimodal imaging in PV IE. Methods: This is a single-center case series. We retrospectively analyzed demographic, laboratory, imaging, clinical, and surgical data from patients diagnosed with PV IE from 2008 to 2018. Results: A total of 23 patients were identified with definite PV IE by Duke criteria (83% male and ages 2 months to 70 years). Twenty-two patients had congenital heart disease, with 21 involving the right ventricular outflow tract (including three with transcatheter PV implant). Overall, 20 (87%) of 23 had positive blood cultures. A total of 17 (74%) of 23 patients demonstrated echo evidence of PV IE. In three cases, echo was negative (did not show vegetations) but showed new PV obstruction. In four cases with negative transthoracic echocardiogram and transesophageal echocardiogram, evidence of PV IE was subsequently seen by positron emission tomography/computed tomography (n = 2) or cardiac magnetic resonance imaging (n = 2). Pulmonary valve IE was confirmed at surgery by evaluation of pathologic samples in 20 cases. Conclusions: Multimodal imaging improves the ability to preoperatively identify endocardial involvement in PV IE in cases where echo is negative. Consideration should be given to revise Duke criteria to include new obstruction and endocardial involvement by multimodal imaging for PV IE.

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