DIAGNOSIS AND TREATMENT: MANAGEMENT OF VENTRICULAR SEPTAL DEFECT

PEDIATRICS ◽  
1964 ◽  
Vol 34 (2) ◽  
pp. 271-273
Author(s):  
Alexander S. Nadas

Intelligent management of a ventricular septal defect necessitates full analysis of the size of the defect, the shunt across it and the status of the pulmonary vasculature. Only patients with moderate or large defects with appreciably increased pulmonary blood flow deserve surgery. Those with small left-to-right shunts, with or without pulmonary vascular disease, should be managed medically.

2001 ◽  
Vol 11 (4) ◽  
pp. 420-430 ◽  
Author(s):  
Elisabeth V. Stenbøg ◽  
Daniel A. Steinbrüchel ◽  
Anne Bloch Thomsen ◽  
Ulrik Baandrup ◽  
Lene Heickendorff ◽  
...  

Introduction: Hypertension and hyperperfusion of the pulmonary vascular bed in the setting of congenital cardiac malformations may lead to progressive pulmonary vascular disease. To improve the understanding of the basic mechanisms of this disease, there is a need for clinically relevant animal models which reflect the disease process. Material and Results: We randomly allocated 45 newborn pigs, at the age of 48 hrs, to groups in which there was either construction of a 3 mm central aorto-pulmonary shunt, undertaken in 9, or ligation of the left pulmonary artery, achieved in 13. Controls included sham operations in 13, or no operations in 10 pigs. Follow-up was continued for three months. The interventions were compatible with survival in most pigs. The shunts resulted in an acute 85% increase in systolic pulmonary arterial pressure, and a more than twofold increase in pulmonary blood flow. By three months of age, nearly all shunts had closed spontaneously, and haemodynamics were normal. Ligation of the left pulmonary artery resulted in a normal total pulmonary blood flow, despite only the right lung being perfused, and a 33% increase in systolic pulmonary arterial pressure. These haemodynamic changes were maintained throughout the period of study. In both groups, histomorphometry revealed markedly increased muscularity of the intra-acinar pulmonary arteries. Circulating levels of endothelin were normal in the shunted animals, and elevated in those with ligation of the left pulmonary artery. Conclusion: In neonatal porcine models of pulmonary vascular disease, created by construction of 3 mm central aorto-pulmonary shunts and ligation of one pulmonary artery, we observed histopathological changes of the pulmonary vasculature similar to early hypertensive pulmonary vascular disease in humans. Elevated circulating levels of endothelin were associated with abnormal haemodynamics rather than abnormal pathology. These findings could be valuable for future studies on the pathogenesis of hypertensive pulmonary vascular disease associated with congenital cardiac malformations.


2007 ◽  
Vol 50 (3) ◽  
pp. 286-292 ◽  
Author(s):  
Matthias Gorenflo ◽  
Michael V Ullmann ◽  
Esther Herpel ◽  
Stephan Neumayer ◽  
Ralf Dieckmann ◽  
...  

2019 ◽  
Vol 29 (7) ◽  
pp. 986-988
Author(s):  
Shyam S. Kothari ◽  
Jay Relan ◽  
Velayoudam Devagourou

AbstractPatients with a significant left-to-right shunt at ventricular level may become inoperable at an early age due to irreversible pulmonary vascular disease. On the other hand, even suprasystemic pulmonary hypertension due to mitral stenosis remains treatable. We report a 24-year-old patient with large ventricular septal defect, severe mitral stenosis and cyanosis who improved after surgical correction of both the lesions. This emphasises the importance of additional post-capillary pulmonary hypertension in Eisenmenger syndrome.


Circulation ◽  
2008 ◽  
Vol 118 (suppl_18) ◽  
Author(s):  
Osami Honjo ◽  
Osman O Al-Radi ◽  
Cathy MacDonald ◽  
Lisa Davey ◽  
Christopher A Caldarone ◽  
...  

OBJECTIVE: We hypothesized mean pulmonary artery (PA) pressure obtained from an intraoperative pulmonary flow study would better predict the ability to close the ventricular septal defect (VSD) and better predict postoperative right ventricular systolic pressure (RSVP) than classic anatomical parameters after complete uniforcalization in patients with pulmonary atresia, VSD, and major aortopulmonary collaterals. METHODS: Sixteen consecutive patients (median, 11 mo, range 2 mo – 16 yrs) underwent one-stage (75%) or staged (25%) uniforcalization between 1/03 and 8/07. Intraoperative functional pulmonary blood flow study was achieved by inserting an arterial cannula in a reconstructed central PA. Flow was increased to 2.5 L/min/m2 while measuring PA pressure. RVSP and systemic systolic pressure (SBP) were recorded after VSD closure. Total neopulmonary artery index (TNPAI) (MAPCA + native PA index), total incorporated pulmonary vascular segments, and pulmonary segment artery ratio (PSAR) (ratio of incorporated segment to 18) were analyzed. Spearman rank correlation and area under the receiver operator characteristics curve (ROC-AUC) were used. RESULTS: The mean PA pressure on flow study was 21.8+/−6.2 mmHg (range, 11–31). Three patients had a pressure of > 30 mmHg. The VSD was closed in 14 (87%). One patient with flow study mean PA pressure of 25 mmHg had suprasystemic RVSP and underwent intraoperative VSD fenestration. One with a flow study mean PA pressure of 30 mmHg had a prospective fenestrated VSD patch placed. There is a weak negative correlation between TNPAI and the flow study PA pressure (rho=−0.4, p=0.12). The flow study mean PA pressure was correlated with post-repair RVSP (rho=0.72, p=0.0027), and with RVSP/SBP ratio (rho=0.67, p=0.0063). TNPAI, total incorporated segments, and PSAR were not correlated with the postoperative RVSP or RVSP/SBP ratio. Flow study mean PA pressure had the highest sensitivity in predicting VSD closure: ROC-AUC (0.82) vs. TNPAI (0.46), pulmonary segment (0.64), and PSAR (0.64). CONCLUSIONS: Intraoperative pulmonary flow study predicted the ability of VSD closure better than total incorporated segments, TNPAI, and PSAR. Flow study mean PA pressure highly correlated with postoperative RVSP and RVSP/SBP ratio.


PEDIATRICS ◽  
1966 ◽  
Vol 38 (2) ◽  
pp. 220-230 ◽  
Author(s):  
Julien I. E. Hoffman ◽  
Abraham M. Rudolph

Three children with big ventricular septal defects, large pulmonary blood flows, and pulmonary hypertension were catheterized at the ages of 3, 6, and 6 months. Pulmonary vascular resistance was low in two and slightly raised in the other. Recatheterization at the ages of 9, 16, and 26 months, respectively, showed increases of pulmonary vascular resistance in all, and the ventricular septal defects were successfully closed by open-heart operation soon thereafter. In the one child who was recatheterized only after there was clinical evidence of a raised pulmonary vascular resistance, postoperative catheterization showed a progressive rise in pulmonary vascular resistance indicating progressive pulmonary vascular disease. The other two children who were clinically well were recatheterized specifically to try and detect early pulmonary vascular changes and, in contrast, in both of these children pulmonary arterial pressures and vascular resistances have returned to normal after operation. These patients demonstrate that in those at risk of developing pulmonary vascular disease (big ventricular septal defect with high pressures and flows), pulmonary vascular resistance can rise rapidly in early life. In these patients progressive pulmonary vascular disease could be prevented if surgery to lower pulmonary arterial pressure and blood flow is done early enough. Even in patients who appear to be improving, recatheterization is necessary to demonstrate a moderate rise in pulmonary vascular resistance, since a moderate rise is not detectable by current clinical techniques.


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