Inge von Alvensleben, Brian B. Graham, Gustavo A. Balanza, Carlos Brockmann, Ericka C. Pérez, Ximena Y. Vicente, Carla Arteaga, Claudia Scherer, Franz P. Freudenthal, Rubin M. Tuder, Alexandra Heath
Kardiozentrum. University of Colorado. University of California San Francisco. Massachusetts General Hospital and Harvard Medical School. Hospital Belga.
Bolivia and United States
Pulmonary Circulation
Pulm Circ 2025; 15:
DOI: 10.1002/pul2.70049
Abstract
Congenital heart disease (CHD) occurs at increased prevalence at high altitude, but there may be a paradoxical later onset of the development of Eisenmenger syndrome. We hypothesized that congenital heart disease patients at high altitude are protected from an early onset of irreversible pulmonary hypertension. We present a ospective observational case series study, supported by a PVRI grant, of patients in La Paz, Bolivia, located at 3600 m, who underwent surgery for CHD. 10 consecutive patients aged 5 to 29 years (mean 12) with left-to-right shunts and pulmonary hypertension underwent diagnostic catheterization to assess pulmonary pressure pre-operatively and six to 9 months postoperatively, and had a lung biopsy performed at the time of the surgery. Control lung tissue was obtained from patients living at the same altitude who underwent pulmonary hydatid cyst resection. 10 CHD patients and 4 control patients were analyzed. Pre-operatively, the patients had a significant response to hyperoxia with a fall in mean pulmonary pressure (mPAP) from 59.6 mmHg (SD 7.74) to 46.3 mmHg (SD 11.1); (p < 0.05). Postoperatively, the patients had an excellent response to surgery, with a mPAP of 26.4 mmHg (SD 6.42) (p < 0.05 vs. preoperative pressures). Analysis of the lung histology did not show evidence of pulmonary vascular remodeling in the CHD patients compared to the control patients. During the follow-up in up to 11 years, pulmonary pressure assessed by echocardiography remained normal. In conclusion, chronic hypobaric hypoxemia at high altitude may delay the development of pulmonary vascular disease in CHD patients.
Category
Class I. Pulmonary Hypertension Associated with Congenital Cardiovascular Disease
Class III. Pulmonary Hypertension Associated with Alveolar Hypoxia
Diagnostic Testing for Pulmonary Vascular Disease. Invasive Testing
Age Focus: Pediatric Pulmonary Vascular Disease or Adult Pulmonary Vascular Disease
Fresh or Filed Publication: Fresh (PHresh). Less than 1-2 years since publication
Article Access
Free PDF File or Full Text Article Available Through PubMed or DOI: Yes