Benefits of perioperative sildenafil therapy in children with a ventricular septal defect with pulmonary artery hypertension on early surgical outcomes

2020 ◽  
Vol 35 (12) ◽  
pp. 3302-3309 ◽  
Author(s):  
Ruchit Patel ◽  
Shyam Kumar Singh Thingnam ◽  
Anand Kumar Mishra ◽  
Manoj Kumar Rohit ◽  
Vinay Upadhyay ◽  
...  
Author(s):  
Divya Chauhan ◽  
Rachna Agarwal ◽  
Prerna Gupta ◽  
Gita Radhakrishnan ◽  
Ayalur Gopalakrishnan Radhika

Patients with Eisenmenger syndrome are advised against pregnancy. Sometimes patients are diagnosed with Eisenmenger syndrome for the first time during pregnancy. This is what has happened in our case. A primigravida was diagnosed with ventricular septal defect with severe pulmonary artery hypertension for the first time during pregnancy at 34 weeks period of gestation. Management of such patients is challenging with different studies showing variable results. Here we describe such a case which was managed conservatively and was allowed to go into labour spontaneously with vacuum extraction during second stage which subsequently led to a favourable maternal outcome.


2020 ◽  
pp. 021849232096292
Author(s):  
Abbas Afrasiabirad ◽  
Mahmoud Samadi ◽  
Parisa Vatani ◽  
Yousef Faridvand

Objective This study aimed to show the long-term results in patients who underwent unidirectional valve patch repair of ventricular septal defect with pulmonary artery hypertension. Methods Thirty-five acyanotic patients aged 2 to 26 years (mean 9.3 years) with a large ventricular septal defect and elevated pulmonary vascular resistance (mean 9.5 Wood units) underwent surgery in Madani Heart Hospital. The medical records and clinical outcomes were reviewed from March 1998 to March 2017. Results Five patients died in the first postoperative week. In the long-term follow-up (mean 11 years), two patients were lost to follow-up. Pulmonary artery hypertension gradually decreased in 17 patients within 6–12 months with significant improvement in right ventricular end-diastolic diameter, New York Heart Association functional class, and tricuspid regurgitation. Eleven patients with persistent pulmonary artery hypertension were divided into tolerable and non-tolerable groups. Six patients in the tolerable group had satisfactory conditions compared to before the operation, and gained weight with improved functional class despite echocardiographic findings of persistent elevated pulmonary artery pressure. One had a full-term delivery by caesarean section in the fifth postoperative year. Five patients in the non-tolerable group gradually developed right heart failure and complications such as extremity edema, ascites, pleural effusions, and died after 10–30 months. Conclusion Although relatively high mortality occurred during long-term follow-up, surviving patients were in a better condition and functional class despite persistent pulmonary artery hypertension. Therefore, fear of persistent pulmonary artery hypertension should not prohibit surgery in this group of patients.


2020 ◽  
Vol 30 (6) ◽  
pp. 799-806
Author(s):  
Anna G. Palladino-Davis ◽  
Christopher S. Davis

AbstractBackground:Pulmonary vascular disease resulting from CHDs may be the most preventable cause of pulmonary artery hypertension worldwide. Many children in developing countries still do not have access to early closure of clinically significant defects, and the long-term outcomes after corrective surgery remain unclear. Focused on long-term results after isolated ventricular septal defect repair, our review sought to determine the most effective medical therapy for the pre-operative management of elevated left-to-right shunts in patients with an isolated ventricular septal defect.Methods:We identified articles specific to the surgical repair of isolated ventricular septal defects. Specific parameters included the pathophysiology and pre-operative medical management of pulmonary over-circulation and outcomes.Results:Studies most commonly focused on histologic changes to the pulmonary vasculature and levels of thromboxanes, prostaglandins, nitric oxide, endothelin, and matrix metalloproteinases. Only 2/44 studies mentioned targeted pharmacologic management to any of these systems related to ventricular septal defect repair; no study offered evidence-based guidelines to manage pulmonary over-circulation with ventricular septal defects. Most studies with long-term data indicated a measurable frequency of pulmonary artery hypertension or diminished exercise capacity late after ventricular septal defect repair.Conclusion:Long-term pulmonary vascular and respiratory changes can occur in children after ventricular septal defect repair. Research should be directed at providing an evidenced-based approach to the medical management of infants and children with ventricular septal defects (and naturally all CHDs) to minimise consequences of pulmonary artery hypertension, particularly as defect repair may occur late in underprivileged societies.


PEDIATRICS ◽  
1964 ◽  
Vol 33 (6) ◽  
pp. 995-996
Author(s):  
John W. Kirklin

AMONG the many interesting congenital cardiac malformations amenable to surgery today, four will be discussed, namely, coarctation of the aorta, ventricular septal defect, Tetralogy of Fallot, and transposition of the great vessels. Recommendations for surgical therapy are based on the experience at the Mayo Clinic. Coarctation of the aorta continues to be most satisfactorily treated by operation and the hospital mortality risk is extremely low. In general, the optimal age for surgery is about 10 to 12 years. Unusually severe systemic hypertension or cardiac enlargement constitute an indication for somewhat earlier surgery. When infants with isolated coarctation of the aorta show severe cardiac failure, and this certainly occurs although uncommonly, operation should be advised at that age. The risk even then is very low, but few cases may require reoperation at age 12 because of failure of adequate growth of the anastomosis. The indications for repair of ventricular septal defect must take into account the estimated size of the lesion and its hemodynamic effects. Patients with small ventricular septal defects who have normalsized hearts and normal electrocardiograms, that is, those without clinical evidence of significant hemodynamic derangement, are not advised by us to undergo repair of the defect at present. Patients with ventricular septal defect and mild or moderate pulmonary artery hypertension (that is, patients whose pulmonary artery pressure is less than 75% of systemic artery pressure) can be operated upon at essentially no hospital risk. Operation is clearly advisable in all of these patients. Patients with ventricular septal defect who have severe pulmonary artery hypertension associated with a large pulmonary blood flow (and thus with only mild elevation of pulmonary vascular resistance) can be operated upon with a low hospital mortality rate, in the neighborhood of 5%.


2020 ◽  
pp. 1-9
Author(s):  
Dong Zhao ◽  
Keming Yang ◽  
Wei Feng ◽  
Shoujun Li ◽  
Jun Yan ◽  
...  

Abstract Objective: This study aimed to investigate the association between long-term survival and different management of major aortopulmonary collateral arteries in patients with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries. Methods: From November, 2009 to October, 2018, a total of 98 consecutive patients with pulmonary atresia, ventricular septal defect, major aortopulmonary collateral arteries, and hypoplastic pulmonary arteries treated with modified Blalock–Taussig shunt or right ventricle–pulmonary artery connection were included. Fifty-five patients who received occlusion or ligation of major aortopulmonary collateral arteries during or after palliative procedure were occlusion group, and the other 43 patients were no occlusion group. The early and late outcomes were compared. Results: The mean duration of follow-up was 30.9 months in no occlusion group and 49.8 months in the occlusion group (p < 0.001). Multivariate analysis showed that only no occlusion of major aortopulmonary collateral arteries was predictive of total mortality (Hazard Ratio: 4.42, 95% CI: 1.27 to 15.42, p = 0.02). The Kaplan–Meier survival curves confirmed that patients without occlusion of major aortopulmonary collateral arteries demonstrated worse survival as compared with the occlusion group (p = 0.013). The Kaplan–Meier survival curves of patients who underwent different palliative procedures showed no differences. Conclusions: For patients with pulmonary atresia, ventricular septal defect and major aortopulmonary collateral arteries when a primary repair is not feasible, those without occlusion of major aortopulmonary collateral arteries have a higher risk of death following an initial palliative procedure compared with patients who underwent occlusion of major aortopulmonary collateral arteries. The occlusion of major aortopulmonary collateral arteries is not associated with a higher rate of complete repair or better improvement of pulmonary artery growth.


2000 ◽  
Vol 70 (1) ◽  
pp. 119-123 ◽  
Author(s):  
Zahid Amin ◽  
Doff B McElhinney ◽  
V.Mohan Reddy ◽  
Phillip Moore ◽  
Frank L Hanley ◽  
...  

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