The “wall-to-wall” heart in the patient with pulmonary atresia and intact ventricular septum

Freedom, Robert M.; Jaeggi, Edgar; Perrin, Donald G.; Yoo, Shi‐Joon; Anderson, Robert H.

doi:10.1017/s1047951105002040

Cited by 31 publications

(6 citation statements)

References 81 publications

(122 reference statements)

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“…These patients may account for approximately one-sixth of those with PA-IVS. 33 Additional predictors include indirect assessment of the right atrial pressure using the tricuspid valve, as well as atrial communication and venous duct Doppler. 34 While prenatal diagnosis is certainly invaluable in assisting with early counseling for families, prenatal diagnosis has not been shown to decrease mortality rate.…”

Section: Fetal Diagnosismentioning

confidence: 99%

Pulmonary Atresia With an Intact Ventricular Septum: Preoperative Physiology, Imaging, and Management

Chikkabyrappa

Loomba

Tretter

2018

Semin Cardiothorac Vasc Anesth

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Pulmonary atresia with intact ventricular septum (PA-IVS) is a rare complex cyanotic congenital heart disease with heterogeneous morphological variation. Prenatal diagnosis allows for developing a safe plan for delivery and postnatal management. While transthoracic echocardiography allows for detailed delineation of the cardiac anatomy, additional imaging modalities such as computed tomography, magnetic resonance imaging, and catheterization may be necessary to further outline features of the cardiac anatomy, specifically coronary artery anatomy. The size of the tricuspid valve and right ventricular cavity as well as the presence of right ventricle-dependent coronary circulation help to dichotomize between biventricular repair versus univentricular palliation or heart transplantation, as well as predicting the expected survival. The delineation and understanding of these features help to dictate both medical and surgical management.

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Section: Fetal Diagnosismentioning

confidence: 99%

Pulmonary Atresia With an Intact Ventricular Septum: Preoperative Physiology, Imaging, and Management

Chikkabyrappa

Loomba

Tretter

2018

Semin Cardiothorac Vasc Anesth

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“…PA-IVS is a rare CHD, accounting for 1.7-3.1% of all CHDs [Grossfeld et al, 1997] and having a prevalence of 4.5 per 100,000 live-births [Freedom et al, 2006]. It can be classified into three types according to Choi et al [1998] including cases that present with right ventricular hypertrophy, tricuspid valve dysplasia and a small right ventricular chamber (type I), such as the cases reported here.…”

mentioning

confidence: 73%

Pulmonary atresia with intact ventricular septum (PA‐IVS) in monozygotic twins

Stefano

Xiang

et al. 2008

American J of Med Genetics Pt A

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E. 2008. Pulmonary atresia with intact ventricular septum (PA-IVS) in monozygotic twins. Am J Med Genet Part A 146A:525-528. To the Editor:Pulmonary atresia with intact ventricular septum (PA-IVS) is a rare congenital heart defect (CHD) of unknown etiology, characterized by an atretic pulmonary valve with the right ventricle varying in size and morphology among different affected individuals. We report on monozygotic (monochorionic diamnionic) twin sisters with PA-IVS who died soon after birth. The patients were prematurely born at 27 weeks by caesarean to a 24-year-old gravida 1, para 0 Hispanic mother. The pregnancy course was previously uneventful, and the mother denied any exposures during pregnancy. Both parents were healthy, nonconsanguineous, with no known family history of CHD in a three-generation pedigree. The Apgar scores were 5 and 7 (Twin A), and 4 and 6 (Twin B), at 1 and 5 min, respectively. Birth weight was 1,050 g (60th centile, Twin A) and 910 grams (40th centile, Twin B), crown-heel length was 35 cm for both twins, and occipital-frontal circumferences were 24.8 cm (Twin A) and 25 cm (Twin B). Soon after birth, Twin A developed increasing respiratory rate requiring mechanical ventilation, and a systolic ejection murmur at the left upper sternal border was noted accompanied by poor oxygen saturations. Chest X-ray revealed hazy, granular lung fields consistent with hyaline membrane disease as well as a cardiac silhouette occupying nearly 70% of the thoracic diameter. An echocardiogram revealed an annular-type pulmonary valve atresia, and aortic stenosis with a bicuspid aortic valve. The left ventricle appeared normal in size and function. In spite of therapy, the patient developed respiratory failure and died at the age of 3 days. Twin B had a similar clinical course and the echocardiogram also showed pulmonary valve atresia (without aortic atresia), suprasystemic right ventricular systolic pressures and moderate tricuspid regurgitation. On day 3 of life, the echocardiogram showed poor cardiac function with left ventricular ejection fraction of 45%. Grade IV germinal matrix hemorrhage was detected by ultrasound. Comfort care was provided, and she died on day 7 of life. Unrestricted postmortem examinations, following perinatal autopsy protocols were performed in both patients.The cardiac findings present in both twins were virtually identical, consistent with the diagnosis of PA-IVS, and characterized by cardiomegaly Twin A: 16.5 g; Twin B: 15.2 g), marked right atrial dilatation, marked right ventricular myocardial hypertrophy (8-10 mm in thickness) and a small right ventricular chamber measuring approximately 9 mm from the atrioventricular annulus to the apical aspect of the chamber. In both cases, the tricuspid valves were dysplastic with two identifiable thick leaflets, attached to the right ventricular endocardium through markedly short and thickened chorda tendineae and papillary muscles. The pulmonary valve was completely atretic in both patients, with three fused valves leaving no identifi...

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“…In Ebstein anomaly , isolated or in association with pulmonary atresia with IVS (64), the extreme atrialization of the right ventricle is such that the residual ventricle is unable to sustain the pulmonary circulation if biventricular repair is accomplished, because the download displacement of septal and posterior leaflets of the tricuspid valve reduces the ventricle to apical and outlet portions (Figure 14). …”

Section: Discussionmentioning

confidence: 99%

The New Concept of Univentricular Heart

Frescura

Thiene

2014

Front. Pediatr.

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The concept of univentricular heart moved from hearts with only one ventricle connected with atria [double inlet ventricle or absent atrioventricular (AV) connection] to hearts not amenable to biventricular repair, namely hearts with two ventricles unable to sustain separately pulmonary and systemic circulations in sequence. In the latter definition, even hearts with one hypoplastic ventricle are considered “functional” univentricular hearts. They include pulmonary/aortic atresia or severe stenosis with hypoplastic ventricle, and rare conditions like huge intramural cardiac tumors and Ebstein anomaly with extreme atrialization of right ventricular cavity. In this setting, the surgical repair is univentricular with “Fontan” operation, bypassing the ventricular mass. In other words, functionally univentricular heart is a condition in which, after surgery, only one ventricle sustain systemic circulation. Univentricular hearts (double inlet or absent AV connection) almost invariably show two ventricular chambers, one main and one accessory, which lacks an inlet portion. The latter is located posteriorly when morphologically left and anteriorly when morphologically right. As far as double inlet left ventricle, this is usually associated with discordant ventriculo-arterial (VA) connection (transposition of the great arteries) and all the blood flow to the aorta, which takes origin from the hypoplastic anterior right ventricle, is ventricular septal defect (bulbo-ventricular foramen) dependent. If restrictive, an aortic arch obstruction may be present. Double inlet left ventricle may be rarely associated with VA concordance (Holmes heart). As far as double inlet right ventricle with posterior hypoplastic left ventricular cavity, ventriculo-arterial connection is usually of double outlet type; thus the term double inlet–outlet right ventricle may be coined. Absent right or left AV connection may develop in the setting of both d- or l-loop, whatever the situs. In this condition, the contra-lateral patent AV valve may be either mitral or tricuspid in terms of morphology and the underlying ventricle (main chamber) either morphologically left or right. Establishing the loop, whatever right or left (also called right or left ventricular topology), is a fundamental step in the segmental-sequential analysis of congenital heart disease.

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The “wall-to-wall” heart in the patient with pulmonary atresia and intact ventricular septum

Cited by 31 publications

References 81 publications

Pulmonary Atresia With an Intact Ventricular Septum: Preoperative Physiology, Imaging, and Management

Pulmonary Atresia With an Intact Ventricular Septum: Preoperative Physiology, Imaging, and Management

Pulmonary atresia with intact ventricular septum (PA‐IVS) in monozygotic twins

The New Concept of Univentricular Heart

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