Outcomes of infants and children undergoing surgical repair of ventricular septal defect: a review of the literature and implications for research with an emphasis on pulmonary artery hypertension

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.

ESC CardioMed ◽  
2018 ◽  
pp. 799-801
Author(s):  
Orla Franklin

Ventricular septal defect is the commonest congenital malformation and many are small and close spontaneously. Significant ventricular septal defects require closure in infancy. Long-term outcome is excellent.


2016 ◽  
Vol 27 (1) ◽  
pp. 181-183
Author(s):  
Shreesha S. Maiya ◽  
Smruti V. Patel ◽  
Chinnaswamy Reddy ◽  
Suresh V. Pujar

AbstractA male child, with d-transposition of great arteries, a large perimembranous ventricular septal defect, multiple additional ventricular septal defects, small muscle-bound right ventricle, and severe pulmonary stenosis with confluent, moderate-sized branch pulmonary arteries, underwent an emergency right modified Blalock–Taussig shunt on day 15 of life and réparation à l’étageventriculaire procedure with ventricular septal defect closure with takedown of the Blalock–Taussig shunt at 2.5 years of age. On follow-up, he showed a moderate residual upper ventricular septal defect and multiple apical ventricular septal defects, mild mid-right pulmonary artery stenosis, free pulmonary regurgitation, and right ventricular dysfunction. Surgical re-intervention was deemed extremely risky, the upper muscular ventricular septal defect was closed using an 8-mm Amplatzer Muscular Ventricular Septal Defect Occluder Device, and an 18 mm Amplatzer Multi-Fenestrated Septal Occluder – Cribriform was used for the multiple apical muscular ventricular septal defects. After 1 year, his right pulmonary artery stenosis worsened, for which right pulmonary artery angioplasty was carried out using an 8×20 mm cutting balloon followed by a 10×20 mm Tyshak II balloon. This is the only case reported for the paediatric age group using a cribriform septal occluder device for percutaneous closure of multiple apical ventricular septal defects.


2003 ◽  
Vol 11 (3) ◽  
pp. 213-216 ◽  
Author(s):  
M Kemal Demirag ◽  
Hasan Tahsin Keçeligil ◽  
Fersat Kolbakir

Between January 1983 and December 2000, 78 patients underwent primary repair of a ventricular septal defect. There were 42 males (54%) and 36 females (46%) of whom 13 (17%) were under 1 year old, 50 (64%) were aged 1–10 years, 11 (14%) were aged 10–20 years, and 4 (5%) were over 20 years old. The ventricular septal defect was a perimembranous type in 60 patients (77%), subarterial (outlet) type in 10 (13%), and atrioventricular canal (inlet) type in 4 (5%). Operative repair was performed with a patch in all except 2 patients. Early postoperative complications included insignificant aortic regurgitation in 4 patients, persistent complete heart block in 1, and residual shunt in 4. There were 5 early deaths (6.4%) and 1 late death (1.8%) in 56 patients followed up. Early primary closure of ventricular septal defects, usually via a right atriotomy, can be performed with acceptable mortality and morbidity rates.


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.


PEDIATRICS ◽  
1989 ◽  
Vol 83 (2) ◽  
pp. 200-203
Author(s):  
Gerard R. Martin ◽  
Lowell W. Perry ◽  
Charlotte Ferencz

The Baltimore-Washington Infant Study is an ongoing case-control study of congenital cardiovascular malformations in infants in whom the clinical diagnoses have been confirmed by echocardiography, catheterization, surgery, or autopsy. An increase in the prevalence of ventricular septal defects was detected in 1,494 infants with congenital cardiovascular malformations between 1981 and 1984. The prevalence of congenital cardiovascular malformations increased from 3.6 to 4.5 per 1,000 live births (P<.025) and the prevalence of ventricular septal defect increased from 1.0 to 1.6 per 1,000 live births (P< .001). The increase in ventricular septal defects accounted for the total increase in congenital cardiovascular malformations. The prevalence of isolated ventricular septal defect increased from 0.67 to 1.17 per 1,000 live births (P<.001). The prevalence of ventricular septal defect with associated coarctation of the aorta, patent ductus arteriosus, atrial septal defect, and pulmonic stenosis did not change. The prevalence of ventricular septal defect diagnosed by catheterization, surgery, and autopsy did not change; however, defects diagnosed by echocardiography increased from 0.30 to 0.70 per 1,000 live births (P<.001). It is concluded that the reported increase in prevalence of ventricular septal defect is due to improved detection of small, isolated ventricular septal defects and that there is no evidence of an "epidemic."


2020 ◽  
Author(s):  
Long Wang ◽  
Lin Xie ◽  
Weiqiang Ruan ◽  
Tao Li ◽  
Changping Gan ◽  
...  

Abstract Background: This report presents updated data and mid-term follow-up information to a former study introducing the novel technique of percutaneous-perventricular device closure of doubly committed subarterial ventricular septal defect. Methods: Thirty-eight patients were added to the former series. There were 54 patients in total who had isolated doubly committed subarterial ventricular septal defects and underwent percutaneous-perventricular device closure. Closure outcomes and possible complications were measured in the hospital and during the 2.5-year follow-up. Results: Surgery was successful in 53 patients (98.1%). There was no death, residual shunt, new valve regurgitation or arrhythmia either perioperatively or during the entire follow-up period. Only one patient developed pericardial effusion and tamponade in the former series. The mean hospital stay was 3.2±0.6 days (range, 3.0 to 6.0 days), and only one unsuccessful case needed blood transfusion (1.9%). Conclusions: The percutaneous-perventricular device closure of isolated doubly committed subarterial ventricular septal defects appeared to be safe. Close monitoring for bleeding is essential postoperatively, especially in younger patients. This technique is generally safe with acceptable mid-term follow-up.


2020 ◽  
Vol 15 (1) ◽  
Author(s):  
Atsushi Morishita ◽  
Ikuo Hagino ◽  
Hideyuki Tomioka ◽  
Seiichiro Katahira ◽  
Takeshi Hoshino ◽  
...  

Abstract Background Partial anomalous pulmonary venous connection draining into the right atrium with an intact atrial septum is a very rare clinical entity in the adult population. Partial anomalous pulmonary venous connection must be suspected as a differential diagnosis when the cause of right heart enlargement and pulmonary artery hypertension is unknown. Case presentation This study describes the surgical case of an isolated right partial anomalous pulmonary venous connection to the right atrium in a 68-year-old woman, who underwent tricuspid ring annuloplasty and right-sided maze procedure simultaneously. She had complaints of gradually progressing dyspnea on exertion. However, a diagnosis could not be established despite consultations at multiple hospitals for over a year. Right heart catheterization revealed severe pulmonary artery hypertension with a mean pulmonary artery pressure of 46 mmHg, step-up phenomenon of oxygen saturation at the mid-level of the right atrium with a pulmonary-to-systemic blood flow ratio of 2.4, and a pulmonary vascular resistance of 3.1 Wood Units. As medical treatment with pulmonary artery vasodilator therapy did not improve her symptoms, she underwent surgical repair. An atrial septal defect was created surgically with a curvilinear tongue-shaped cut. The right anomalous pulmonary veins were rerouted through the surgically created atrial septal defect into the left atrium with a baffle comprised of the interatrial septum flap, kept in continuity with the anterior margin and sutured while mobilizing the enlarged right atrium. The patient had an uneventful postoperative course and remains asymptomatic. Conclusions The described surgical technique could be considered an effective alternative for patients undergoing surgical repair for a partial anomalous pulmonary venous connection isolated to the right atrium. The indication for surgery must be judged on a case-by-case basis in these patients with prevalent systemic-to-pulmonary shunting.


2019 ◽  
Vol 27 (7) ◽  
pp. 529-534
Author(s):  
Noor Mohammad Noori ◽  
Alireza Teimouri

Background Ventricular septal defect, the most common congenital heart defect, is characterized by an opening between the ventricles. This study aimed to evaluate the clinical course and associations between the characteristics of ventricular septal defect. Methods This cross-sectional study was conducted on 1498 children with ventricular septal defects, aged <19 years, who were referred to our center between 2003 and 2018. The diagnosis was suspected from a combination of clinical and laboratory findings, and confirmed by transthoracic echocardiography. Results Of the 1498 children, 54.9% were boys, 78.4% of defects were perimembranous, 30.4% of patients had pulmonary hypertension, 67.5% had regular follow-up, and 76 (5.1%) had complications including 28 (1.9%) with Eisenmenger syndrome; 10 died due to Eisenmenger syndrome during follow-up. The defects closed spontaneously in 38.9% and after surgery in 20.9%. Boys tended to have more perimembranous, inlet, and outlet forms (56.40%, 50.60%, 51.90%, respectively), whereas girls had more muscular types (51.80%). Most patients with pulmonary hypertension had perimembranous defects (83.10%). The majority of patients with pulmonary hypertension had large (63.40%) or moderate (36.60%) defects. Pulmonary hypertension had a significant association with defect size ( p < 0.001). After surgery, residual defects were found mostly in cases of large defects (84.60%). Most patients with spontaneous closure were younger than 4 years. Conclusion Almost four-fifths of children with ventricular septal defects had perimembranous types, and almost one-third had pulmonary hypertension which was associated with defect size. Two-fifths of the defects closed spontaneously. There was a low incidence of complications after surgery.


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