High Altitude May Protect Against the Early Development of Irreversible Pulmonary Hypertension in Patients With Congenital Heart Disease
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 pu...
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Wiley
2025-01-01
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| Series: | Pulmonary Circulation |
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| Online Access: | https://doi.org/10.1002/pul2.70049 |
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| author | Inge vonAlvensleben 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 |
| author_facet | Inge vonAlvensleben 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 |
| author_sort | Inge vonAlvensleben |
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| description | 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. |
| format | Article |
| id | doaj-art-73b44ff35a094d02ba3e6fb24875c2aa |
| institution | OA Journals |
| issn | 2045-8940 |
| language | English |
| publishDate | 2025-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Pulmonary Circulation |
| spelling | doaj-art-73b44ff35a094d02ba3e6fb24875c2aa2025-08-20T02:10:28ZengWileyPulmonary Circulation2045-89402025-01-01151n/an/a10.1002/pul2.70049High Altitude May Protect Against the Early Development of Irreversible Pulmonary Hypertension in Patients With Congenital Heart DiseaseInge vonAlvensleben0Brian B. Graham1Gustavo A. Balanza2Carlos Brockmann3Ericka C. Pérez4Ximena Y. Vicente5Carla Arteaga6Claudia Scherer7Franz P. Freudenthal8Rubin M. Tuder9Alexandra Heath10Department of Pediatric Cardiology Kardiozentrum La Paz BoliviaDivision of Pulmonary Medicine University of Colorado Denver Colorado USADepartment of Anesthesiology, Critical Care and Pain Medicine MGH, Harvard Medical School Boston Massachusetts USADepartment of Cardiac Surgery Hospital Belga Cochabamba BoliviaDepartment of Cardiac Surgery Hospital Belga Cochabamba BoliviaDepartment of Pediatric Cardiology Kardiozentrum La Paz BoliviaDepartment of Pediatric Cardiology Kardiozentrum La Paz BoliviaDepartment of Pediatric Cardiology Kardiozentrum La Paz BoliviaDepartment of Pediatric Cardiology Kardiozentrum La Paz BoliviaDivision of Pulmonary Medicine University of Colorado Denver Colorado USADepartment of Pediatric Cardiology Kardiozentrum La Paz BoliviaABSTRACT 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.https://doi.org/10.1002/pul2.70049chronic hypobaric hypoxemiacongenital heart defectEisenmenger syndromehigh altitudelung biopsy in cardiac shunts |
| spellingShingle | Inge vonAlvensleben 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 High Altitude May Protect Against the Early Development of Irreversible Pulmonary Hypertension in Patients With Congenital Heart Disease Pulmonary Circulation chronic hypobaric hypoxemia congenital heart defect Eisenmenger syndrome high altitude lung biopsy in cardiac shunts |
| title | High Altitude May Protect Against the Early Development of Irreversible Pulmonary Hypertension in Patients With Congenital Heart Disease |
| title_full | High Altitude May Protect Against the Early Development of Irreversible Pulmonary Hypertension in Patients With Congenital Heart Disease |
| title_fullStr | High Altitude May Protect Against the Early Development of Irreversible Pulmonary Hypertension in Patients With Congenital Heart Disease |
| title_full_unstemmed | High Altitude May Protect Against the Early Development of Irreversible Pulmonary Hypertension in Patients With Congenital Heart Disease |
| title_short | High Altitude May Protect Against the Early Development of Irreversible Pulmonary Hypertension in Patients With Congenital Heart Disease |
| title_sort | high altitude may protect against the early development of irreversible pulmonary hypertension in patients with congenital heart disease |
| topic | chronic hypobaric hypoxemia congenital heart defect Eisenmenger syndrome high altitude lung biopsy in cardiac shunts |
| url | https://doi.org/10.1002/pul2.70049 |
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