Primary pulmonary hypertension in children living at high altitude

Khoury, George H.; Hawes, Charles R.

doi:10.1016/s0022-3476(63)80389-3

Cited by 54 publications

(16 citation statements)

References 26 publications

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“…Pathologic findings included medial hypertrophy of small pulmonary arteries, muscularization of pulmonary arterioles, and severe right ventricular hypertrophy and dilation. A similar clinical syndrome was reported in 5 infants and 6 older children from Leadville, Colorado, at 3100 m (Khoury and Hawes, 1963). Three of these children had pulmonary hypertension at cardiac catheterization, and one had medial hypertrophy and intimal thickening at autopsy.…”

Section: Niermeyersupporting

confidence: 71%

Cardiopulmonary Transition in the High Altitude Infant

Niermeyer

2003

High Altitude Medicine & Biology

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The perinatal cardiopulmonary transition at high altitude differs from that at sea level because oxygen plays a fundamental role in the developmental changes from fetus to newborn infant. Under conditions of high altitude hypoxia, arterial oxygen saturations are lower, breathing patterns and maturation of respiratory control reflexes differ, and regression of fetal characteristics of the pulmonary vasculature proceeds more slowly. Several aspects of transition vary not only with postnatal age and altitude, but also with population group, suggesting an effect of genetic adaptation on perinatal physiology. Exposure to chronic high altitude hypoxia during the perinatal transition also results in apparent lifelong alterations in respiratory reflex responses and pulmonary vasoreactivity. Disruption of the normal process of cardiopulmonary transition can result in symptomatic high altitude pulmonary hypertension. The exaggerated hypoxemia associated with acute respiratory infections in young infants still undergoing transition contributes to infant mortality at high altitude.

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Section: Niermeyersupporting

confidence: 71%

Cardiopulmonary Transition in the High Altitude Infant

Niermeyer

2003

High Altitude Medicine & Biology

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“…SHAPH most commonly afflicts infants but also children, primarily offspring of Chinese of Han ancestry who have moved from low altitude to the Qinghai-Tibet Plateau. SHAPH most frequently occurs above 3000 m. 556 There are sporadic reports of SHAPH outside Tibet, including reports from La Paz, Bolivia, 557 and Leadville, CO. 558 Unlike patients with Monge disease, patients with SHAPH do not present with polycythemia. Presenting symptoms of SHAPH are consistent with congestive heart failure.…”

Section: Symptomatic High-altitude Phmentioning

confidence: 99%

Pediatric Pulmonary Hypertension

Abman¹,

Hansmann²,

Archer³

et al. 2015

Circulation

949

405

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Abstract-Pulmonary hypertension is associated with diverse cardiac, pulmonary, and systemic diseases in neonates, infants, and older children and contributes to significant morbidity and mortality. However, current approaches to caring for pediatric patients with pulmonary hypertension have been limited by the lack of consensus guidelines from experts in the field. In a joint effort from the American Heart Association and American Thoracic Society, a panel of experienced clinicians and clinician-scientists was assembled to review the current literature and to make recommendations on the diagnosis, evaluation, and treatment of pediatric pulmonary hypertension. This publication presents the results of extensive literature reviews, discussions, and formal scoring of recommendations for the care of children with pulmonary hypertension. Key Words: AHA Scientific Statements ◼ bronchopulmonary dysplasia ◼ congenital diaphragmatic hernia ◼ congenital heart disease ◼ genetics ◼ persistent pulmonary hypertension of the newborn ◼ sickle cell disease © 2015 by the American Heart Association, Inc., and the American Thoracic Society.Circulation is available at http://circ.ahajournals.org DOI: 10.1161/CIR.0000000000000329 †Deceased. The American Heart Association and the American Thoracic Society make every effort to avoid any actual or potential conflicts of interest that may arise as a result of an outside relationship or a personal, professional, or business interest of a member of the writing panel. Specifically, all members of the writing group are required to complete and submit a Disclosure Questionnaire showing all such relationships that might be perceived as real or potential conflicts of interest.This document was approved by the American Heart Association Science Advisory and Coordinating Committee on May 12, 2015, the American Heart Association Executive Committee on July 22, 2015, and the American Thoracic Society on July 24, 2015.The online-only Data Supplement is available with this article at http://circ.ahajournals.org/lookup/suppl/doi:10.1161/CIR.0000000000000329/-/DC1. The American Heart Association requests that this document be cited as follows: Abman SH, Hansmann G, Archer SL, Ivy DD, Adatia I, Chung WK, Hanna BD, Rosenzweig EB, Raj JU, Cornfield D, Stenmark KR, Steinhorn R, Thébaud B, Fineman JR, Kuehne T, Feinstein JA, Friedberg MK, Earing M, Barst RJ, Keller RL, Kinsella JP, Mullen M, Deterding R, Kulik T, Mallory G, Humpl T, Wessel DL; on behalf of the American Heart Association Council on Cardiopulmonary, Critical Care, Perioperative and Resuscitation, Council on Clinical Cardiology, Council on Cardiovascular Disease in the Young, Council on Cardiovascular Radiology and Intervention, Council on Cardiovascular Surgery and Anesthesia, and the American Thoracic Society. Pediatric pulmonary hypertension: guidelines from the American Heart Association and American Thoracic Society. Circulation. 2015;132:2037-2099 Copies: This document is available on the World Wide Web site of the American Heart Associat...

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“…57 There is also a greater frequency of symptomatic pulmonary hypertension (subacute infantile mountain sickness), persistent patent ductus arteriosus, and PFO later in childhood at high than at low altitude. 37,41,[66][67][68][69][70][71][72] While several reports indicate that population ancestry influences resting P pa and the pulmonary vasoconstrictor response to hypoxia during adulthood (Fig. 1B), little is known concerning population variation in P pa during infancy or childhood.…”

Section: Discussionmentioning

confidence: 99%

Neonatal Oxygenation, Pulmonary Hypertension, and Evolutionary Adaptation to High Altitude (2013 Grover Conference series)

Niermeyer

Andrade-M²,

Vargas

et al. 2015

Pulm. circ.

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Andeans and Tibetans have less altitude reduction in birth weight than do shorter-resident groups, but only Tibetans are protected from pulmonary hypertension and chronic mountain sickness (CMS). We hypothesized that differences in neonatal oxygenation were involved, with arterial O 2 saturation (SaO 2 ) being highest in Tibetans, intermediate in Andeans, and lowest in Han or Europeans, and that improved oxygenation in Andeans relative to Europeans was accompanied by a greater postnatal decline in systolic pulmonary arterial pressures (P pa sys ). We studied 41 healthy (36 Andeans, 5 Europeans) and 9 sick infants at 3,600 m in Bolivia. The SaO 2 in healthy babies was highest at 6-24 hours of postnatal age and then declined, whereas sick babies showed the opposite pattern. Compared to that of 30 Tibetan or Han infants studied previously at 3,600 m, SaO 2 was higher in Tibetans than in Han or Andeans during wakefulness and active or quiet sleep. Tibetans, as well as Andeans, had higher values than Han while feeding. The SaO 2 's of healthy Andeans and Europeans were similar and, like those of Tibetans, remained at 85% or above, whereas Han values dipped below 70%. Andean and European P pa sys values were above sea-level norms and higher in sick than in healthy babies, but right heart pressure decreased across 4-6 months in all groups. We concluded that Tibetans had better neonatal oxygenation than Andeans at 3,600 m but that, counter to our hypothesis, neither was SaO 2 higher nor P pa lower in Andean than in European infants. Further, longitudinal studies in these 4 groups are warranted to determine whether neonatal oxygenation influences susceptibility to high-altitude pulmonary hypertension and CMS later in life.Keywords: Andean, cardiopulmonary transition, Ethiopian, European, genetic adaptation, Han, hypoxia, Tibet. Studies at high altitude have long provided a useful model for persons interested in the pulmonary circulation. [1][2][3][4] While pulmonary arterial pressures (P pa 's) are generally elevated at high altitude, there is considerable population variation in both P pa and pulmonary circulation-related disorders such as chronic mountain sickness (CMS; Fig. 1A) 11,36 and subacute infantile mountain sickness. 37 Specifically, directly measured P pa and hypoxic pulmonary vasoconstrictor responses are lowest in Tibetans, intermediate in Andeans, and highest in European-derived residents of Colorado (Fig. 1B). Recent estimates using echocardiography also suggest intermediate values in Ethiopians. 15 While multiple factors contribute to the etiology of CMS and no well-controlled comparative epidemiological studies exist, a reduced prevalence among Tibetans is consistent with the lower hemoglobin levels seen in Tibetans, relative to Andeans, in multiple reports. [38][39][40] Tibetans are also protected, relative to Han, from subacute infantile mountain sickness, a syndrome characterized by severe pulmonary hypertension in 4-6-month-old infants that has also been reported in European-derived popul...

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Primary pulmonary hypertension in children living at high altitude

Cited by 54 publications

References 26 publications

Cardiopulmonary Transition in the High Altitude Infant

Cardiopulmonary Transition in the High Altitude Infant

Pediatric Pulmonary Hypertension

Neonatal Oxygenation, Pulmonary Hypertension, and Evolutionary Adaptation to High Altitude (2013 Grover Conference series)

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