Management evolution of pulmonary atresia and intact ventricular septum

Leung, Maurice P.; Mok, C. K.; Lee, Jan; Lo, Roxy N.S.; Cheung, Henry; Chiu, Chia-Yu

doi:10.1016/0002-9149(93)90550-v

Cited by 24 publications

(18 citation statements)

References 17 publications

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“…Traditionally, the RV morphological and coronary artery anatomic variables were believed to be the major determinants of outcomes for the patients with PAIVS [11,17,18,19]. However, these seemed unable to completely explain the overall poor prognosis of this disease.…”

Section: Discussionmentioning

confidence: 99%

Decreased Left Ventricular Coronary Artery Density in Pulmonary Atresia and Intact Ventricular Septum

2007

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Background: The prognosis for pulmonary atresia and intact ventricular septum (PAIVS) has been poor. Our hypothesis is that intrinsic abnormal left ventricular (LV) intramyocardial circulation might be related to the poor outcomes of these patients. Methods:Neonatal heart specimens were examined microscopically in four groups of 6 cases each. Group I had PAIVS with ventriculocoronary artery connections (VCAC), group II had PAIVS without VCAC, group III had normal hearts, and group IV had LV hypertrophy. A projection microscope with grid overlay was used to count the LV intramyocardial coronary artery density (IMCAD), which was expressed as the number of profiles/mm². Results: The LV IMCAD of groups I (0.40 ± 0.14/mm²) and II (0.45 ± 0.15/mm²) were significantly lower than those of groups III (0.77 ± 0.11/mm²) and IV (0.76 ± 0.09/mm²; all with p = 0.002). There was no significant difference between either groups I and II (p = 0.394) or groups III and IV (p = 0.818). Conclusions: This study demonstrates lower LV IMCAD in a widely heterogeneous spectrum of neonatal hearts with PAIVS, which might potentially predispose these patients to myocardial ischemia and in turn contribute to the poor prognosis of this disease.

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Section: Discussionmentioning

confidence: 99%

Decreased Left Ventricular Coronary Artery Density in Pulmonary Atresia and Intact Ventricular Septum

2007

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“…Since the beginning, all surgical efforts and attempts have undergone several changes; however, the selection of patients for surgery has always been based on the size of the right ventricular cavity, anatomy and function of the tricuspid valve, and the presence of coronary-cavitary connections 3,[19][20][21][22][23][24][25][26][27][28][29][30] . The following surgical techniques have been used: valvotomy with hypothermia or cardioplegia with cardiopulmonary by-pass; pulmonary valvectomy; and transannular patch 3,[19][20][21][22][23][24][25][26][27][28][29][30] .…”

mentioning

confidence: 99%

“…The following surgical techniques have been used: valvotomy with hypothermia or cardioplegia with cardiopulmonary by-pass; pulmonary valvectomy; and transannular patch 3,[19][20][21][22][23][24][25][26][27][28][29][30] . With the development of interventional techniques for the pediatric patient, a new alternative for the treatment of this malformation appeared with cardiac catheterization.…”

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confidence: 99%

Características morfológicas angiográficas na atresia pulmonar com septo interventricular íntegro

Santos

Azevedo²

2004

Arq. Bras. Cardiol.

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“…1): One prototype is a more frequent type, with pulmonary valvular atresia, dysplasia of the tricuspid valve (TV) with a small annulus, underdeveloped RV with a hypoplastic cavity and a hypertrophic wall, atrial septal defect, and patent ductus arteriosus; the other, less frequent type has more severe dysplasia of TV and dilatation of the RV, right atrium (RA), and right atrioventricular junction, with marked thinning of the RV wall. Although the clinical, angiocardiographic, and pathologic profile of the patient with PAIVS is well established [2,19,26,28], the treatment of these infants, who are often critically ill, continues to pose a considerable challenge [1,9,23,30]. Because the majority of these patients have gross underdevelopment of the RV and inadequate pulmonary bloodflow, surgical intervention is directed toward augmenting pulmonary bloodflow and allowing the potential for right ventricular growth by establishing continuity between the RV and the pulmonary artery, and by the construction of a systemic to pulmonary artery anastomosis [1,9,10,19,22,23,25].…”

mentioning

confidence: 99%

“…Although the clinical, angiocardiographic, and pathologic profile of the patient with PAIVS is well established [2,19,26,28], the treatment of these infants, who are often critically ill, continues to pose a considerable challenge [1,9,23,30]. Because the majority of these patients have gross underdevelopment of the RV and inadequate pulmonary bloodflow, surgical intervention is directed toward augmenting pulmonary bloodflow and allowing the potential for right ventricular growth by establishing continuity between the RV and the pulmonary artery, and by the construction of a systemic to pulmonary artery anastomosis [1,9,10,19,22,23,25]. However, too frequently, adequate continuity between the RV and pulmonary artery is not accomplished, and adequate growth of the RV is seldom achieved, although such growth has been documented in patients with pulmonary stenosis and a small RV [12,27].…”

mentioning

confidence: 99%

Morphology of Tricuspid Valve in Pulmonary Atresia with Intact Ventricular Septum

et al. 1998

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Pulmonary atresia with intact ventricular septum (PAIVS) is a rare congenital cardiac anomaly that has been classified into two types: one is a more frequent type having dysplasia of tricuspid valve (TV) with a small annulus, underdeveloped right ventricle (RV) with a hypoplastic cavity and a hypertrophic wall; the other type has severe dysplasia of TV and dilatation of RV, right atrium (RA), and right atrioventricular junction with thinning of the RV wall. We performed a morphologic study on 11 autopsied hearts with PAIVS, giving particular emphasis to the variation of morphology of the TV. We could classify these hearts into 3 groups according to the degree of right ventricular development. In the first group of 7 cases (type I), the RVs were underdeveloped. Thick leaflets, restricted valve apparatus with short chordae, and small annuli were characteristics of the TV. In the second group of 3 cases (type II), the RVs showed marked enlargement of the cavity and thinning of the wall. The TV showed redundant, dysplastic, sail-like anterior leaflets, and the downward displacement of septal leaflet and/or posterior leaflet, which are the findings frequently observed in Ebstein's malformation. The RVs were dilated and with partially unguarded tricuspid orifice. The septal leaflet of the TV was dysplastic and, in two cases, the septal leaflet showed chordal structure at the upper surface facing the RA, which is a peculiar finding that has not been described in the literature. The remaining case was a heart with a moderately developed RV (type III). The TV showed mildly dysplastic appearance and we classify this as a separate type, because we could expect the best surgical results in this type. This type had optimal size of RV and the mildest degree of dysplasia of TV. In PAIVS, the morphology of TV correlates well with the type of the right ventricular development.

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Management evolution of pulmonary atresia and intact ventricular septum

Cited by 24 publications

References 17 publications

Decreased Left Ventricular Coronary Artery Density in Pulmonary Atresia and Intact Ventricular Septum

Decreased Left Ventricular Coronary Artery Density in Pulmonary Atresia and Intact Ventricular Septum

Características morfológicas angiográficas na atresia pulmonar com septo interventricular íntegro

Morphology of Tricuspid Valve in Pulmonary Atresia with Intact Ventricular Septum

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