Operable ventricular septal defect despite severe pulmonary hypertension and cyanosis!

2019 ◽  
Vol 29 (7) ◽  
pp. 986-988
Author(s):  
Shyam S. Kothari ◽  
Jay Relan ◽  
Velayoudam Devagourou
Keyword(s):  
Pulmonary Hypertension ◽  
Ventricular Septal Defect ◽  
Vascular Disease ◽  
Mitral Stenosis ◽  
Septal Defect ◽  
The Other ◽  
Pulmonary Vascular Disease ◽  
Early Age ◽  
Eisenmenger Syndrome ◽  
Severe Pulmonary Hypertension

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.

INCREASING PULMONARY VASCULAR RESISTANCE DURING INFANCY IN ASSOCIATION WITH VENTRICULAR SEPTAL DEFECT

PEDIATRICS ◽  
1966 ◽  
Vol 38 (2) ◽  
pp. 220-230 ◽  
Author(s):  
Julien I. E. Hoffman ◽  
Abraham M. Rudolph
Keyword(s):  
Ventricular Septal Defect ◽  
Vascular Disease ◽  
Pulmonary Vascular Resistance ◽  
Vascular Resistance ◽  
Septal Defect ◽  
The Other ◽  
Pulmonary Vascular Disease ◽  
Ventricular Septal Defects ◽  
Septal Defects ◽  
Pulmonary Arterial

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.

scholarly journals Heart and lung transplantation in pulmonary arterial hypertension related to congenital heart disease: an unusual indication

2020 ◽  
Vol 4 (S1) ◽  
Author(s):  
Rosaria Barracano ◽  
Heba Nashat ◽  
Andrew Constantine ◽  
Konstantinos Dimopoulos
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Lung Transplantation ◽  
Atrial Septal Defect ◽  
Septal Defect ◽  
Pulmonary Vascular Disease ◽  
Eisenmenger Syndrome ◽  
Pulmonary Arterial ◽  
Recurrent Haemoptysis

Abstract Background Eisenmenger syndrome is a multisystem disorder, characterised by a significant cardiac defect, severe pulmonary hypertension and long-standing cyanosis. Despite the availability of pulmonary hypertension therapies and improved supportive care in specialist centres, Eisenmenger patients are still faced with significant morbidity and mortality. Case presentation We describe the case of a 44-year-old woman with Eisenmenger syndrome secondary to a large secundum atrial septal defect. Her pulmonary vascular disease was treated with pulmonary vasodilators, but she experienced a progressive decline in exercise tolerance, increasing atrial arrhythmias, resulting in referral for transplantation. Her condition was complicated by significant recurrent haemoptysis in the context of extremely dilated pulmonary arteries and in-situ thrombosis, which prompted successful heart and lung transplantation. She made a slow recovery but remains well 3 years post-transplant. Conclusions Patients with Eisenmenger syndrome secondary to a pre-tricuspid lesion, such as an atrial septal defect have a natural history that differs to patients with post-tricuspid shunts; the disease tends to present later in life but is more aggressive, prompting early and aggressive medical intervention with pulmonary arterial hypertension therapies. This case illustrates that severe recurrent haemoptysis can be an indication for expediting transplantation in Eisenmenger syndrome patients.

Pulmonary Vascular Disease in Secundum Atrial Septal Defect with Pulmonary Hypertension

CHEST Journal ◽  
1986 ◽  
Vol 89 (5) ◽  
pp. 694-698 ◽  
Author(s):  
Shigeo Yamaki ◽  
Togo Horiuchi ◽  
Makoto Miura ◽  
Yasuyuki Suzuki ◽  
Eiji Ishizawa ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Atrial Septal Defect ◽  
Vascular Disease ◽  
Septal Defect ◽  
Pulmonary Vascular Disease ◽  
Secundum Atrial Septal Defect

Clinical course in children and adolescents with ventricular septal defect

2019 ◽  
Vol 27 (7) ◽  
pp. 529-534
Author(s):  
Noor Mohammad Noori ◽  
Alireza Teimouri
Keyword(s):  
Pulmonary Hypertension ◽  
Ventricular Septal Defect ◽  
Septal Defect ◽  
Clinical Course ◽  
Defect Size ◽  
Eisenmenger Syndrome ◽  
Ventricular Septal Defects ◽  
Laboratory Findings ◽  
Septal Defects

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.

scholarly journals Late Results after Correction of Ventricular Septal Defect with Severe Pulmonary Hypertension.

1994 ◽  
Vol 174 (1) ◽  
pp. 41-48 ◽  
Author(s):  
KIYOSHI HANEDA ◽  
NAOSHI SATO ◽  
TAKAO TOGO ◽  
MAKOTO MIURA ◽  
MASAKI RATA ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Ventricular Septal Defect ◽  
Septal Defect ◽  
Late Results ◽  
Severe Pulmonary Hypertension
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