Interim Mortality in Pulmonary Atresia With Intact Ventricular Septum

Fenton, Kathleen N.; Pigula, Frank A.; Gandhi, Sanjiv K.; Russo, Linda; Duncan, Kim

doi:10.1016/j.athoracsur.2004.06.015

Cited by 45 publications

(27 citation statements)

References 13 publications

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“…The high incidence of associated intracardiac anomalies, particularly atrioventricular valve regurgitation and conduction abnormalities, in addition to the prevalence of extracardiac malformations in patients with heterotaxy, add to the challenge [12][13][14][15]. Similarly, outcomes of SV patients with PAIVS continue to be unfavorable and multiple studies have identified that PAIVS was an independent risk factor for mortality after mBTS [2,8,16]. In the report examining the STS database, hospital mortality was 7.2% for SV anomalies other than PAIVS and 15.6% for PAIVS [2].…”

Section: Commentmentioning

confidence: 93%

Results of Palliation With an Initial Modified Blalock-Taussig Shunt in Neonates With Single Ventricle Anomalies Associated With Restrictive Pulmonary Blood Flow

Alsoufi

Gillespie

Kogon

et al. 2015

The Annals of Thoracic Surgery

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Section: Commentmentioning

confidence: 93%

Results of Palliation With an Initial Modified Blalock-Taussig Shunt in Neonates With Single Ventricle Anomalies Associated With Restrictive Pulmonary Blood Flow

Alsoufi

Gillespie

Kogon

et al. 2015

The Annals of Thoracic Surgery

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“…Recent large multi-institutional or population-based studies [17][18][19] revealed that the overall survival of PAIVS patients was 57-71% at 1 year and 48-64% at 5 years, and that a successful biventricular repair could only be achieved in about 30%, although the results continue to improve. In addition, Fenton and associates [20] demonstrated a significant interim mortality rate similar to, or somewhat higher than that of hypoplastic left heart syndrome. There must be a group of PAIVS patients who have to undergo a single ventricle repair.…”

Section: Discussionmentioning

confidence: 99%

“…Hausdorf and associates [6] found a high degree of coincidence between regional wall motion abnormalities and myocardial perfusion topography resulting from persisting myocardial sinusoids. Fenton and associates [20] attributed the most common cause of interim mortality to abnormal myocardial perfusion. Others have reported myocardial abnormalities including myocardial noncompaction, disarray [22], and endocardial fibroelastosis [10].…”

Section: Discussionmentioning

confidence: 99%

Exclusion of the non-functioning right ventricle in children with pulmonary atresia and intact ventricular septum☆

Yang¹,

Jun²,

Park³

et al. 2008

European Journal of Cardio-Thoracic Surgery

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Objective A group of patients with pulmonary atresia and intact ventricular septum (PAIVS) have to undergo single ventricle repair. In these patients, the presence of the non-functioning right ventricle (RV) may lead to the aggravation of RV to coronary connections and left ventricular (LV) dysfunction. To prevent these deleterious effects, the RV was excluded surgically. Methods Between December 2000 and February 2006, 10 patients with PAIVS underwent RV exclusion in conjunction with cavo-pulmonary anastomosis (n = 6) or systemic-to-pulmonary artery shunt (n = 4). Median age at surgery was 5 months (range, 0.2-13.8). Median z-value of the tricuspid valve was À4.0 (range, À6.5 to À1.3). None had RV dependent coronary circulation. The tricuspid valve was closed directly or using a patch. Thrombotic materials were inserted into the RV cavity in eight patients. Results There was no mortality. Follow-up was completed in all patients for up to 79.1 months (median, 30.3). Seven patients underwent a Fontan procedure and the other three are waiting. Eight patients showed completely obliterated RV after exclusion. One of the two patients who had residual RV cavity underwent re-exclusion after a Fontan operation. Postoperative echocardiography revealed that LV enddiastolic dimension increased with borderline significance ( p = 0.050), whereas LV end-systolic dimension showed no significant changes. During follow-up, LV showed no evidence of regional ischemia or global dysfunction, and fractional shortening significantly increased compared with the pre-exclusion value ( p = 0.017). Conclusions This study demonstrates that RV exclusion may be performed safely in a selected group of patients with PAIVS. This procedure may have beneficial effects on LV systolic function, by preventing possible ischemia, and on LV diastolic function, by obliterating the non-functioning cavity and improving the geometry of the ventricular septum. #

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“…While the presence of a coronary flow reserve is likely to provide some protection from ischaemia, it is generally thought that patients with PAIVS (and other forms of congenital heart disease) have markedly reduced coronary reserve capacity [58][59][60], which suggests that myocardial flow may be primarily determined by systemic and myocardial factors. However, any reduction in flow seen in this study (without autoregulation) could also be interpreted as a reduction in flow reserve (with autoregulation).…”

Section: Study Limitationsmentioning

confidence: 99%

A numerical model of neonatal pulmonary atresia with intact ventricular septum and RV‐dependent coronary flow

Mynard

Davidson

Penny

et al. 2010

Numer Methods Biomed Eng

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SUMMARYWe developed a numerical model of the neonatal arterial and coronary circulations in the context of pulmonary atresia with intact ventricular septum (PAIVS). PAIVS is a congenital heart defect characterized by a blocked pulmonary valve orifice and elevated right ventricular (RV) pressure, and is often associated with an abnormal connection (fistula) between the RV cavity and a coronary artery (e.g. the left anterior descending artery, LAD). A proximal coronary stenosis is also common and can result in RV-dependent coronary flow, where decompression of the RV by surgical repair of the atresia leads to compromised coronary flow (ischaemia). Using a combined one-dimensional and lumped parameter model, we simulated PAIVS to investigate the effects of an RV-LAD fistula, LAD stenosis and RV decompression on coronary haemodynamics. We also simulated coronary collaterals, which develop in response to inadequate blood flow, and found that these may provide little protection against ischaemia in the setting of PAIVS with RV-LAD fistula and LAD stenosis if collateral flow is preferentially directed to the low-resistance fistula. While the focus of this study was PAIVS, the techniques presented may be applied to a variety of complex congenital heart defects that include abnormal vascular connections and unusual coronary anatomy.

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Interim Mortality in Pulmonary Atresia With Intact Ventricular Septum

Cited by 45 publications

References 13 publications

Results of Palliation With an Initial Modified Blalock-Taussig Shunt in Neonates With Single Ventricle Anomalies Associated With Restrictive Pulmonary Blood Flow

Results of Palliation With an Initial Modified Blalock-Taussig Shunt in Neonates With Single Ventricle Anomalies Associated With Restrictive Pulmonary Blood Flow

Exclusion of the non-functioning right ventricle in children with pulmonary atresia and intact ventricular septum☆

A numerical model of neonatal pulmonary atresia with intact ventricular septum and RV‐dependent coronary flow

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