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Journal article
Portopulmonary hypertension in children: a rare but potentially lethal and under-recognized disease
Pulmonary circulation, v 7(3), pp 712-718
01 Jul 2017
PMCID: PMC5841896
PMID: 28704131
Featured in Collection : UN Sustainable Development Goals @ Drexel
Abstract
Portopulmonary hypertension (PoPH) is defined by the combination of portal hypertension and precapillary pulmonary arterial hypertension (PAH). Very little is known about this process in pediatric patients but prognosis is generally poor. We review our institutional experience and report on five patients with pediatric PoPH. The median age of PoPH diagnosis was six years and PAH was 14 years. PAH diagnosis was made by echocardiogram in all patients, four of whom also had cardiac catheterization. The median mean pulmonary artery pressure (mPAP) was 48.5mmHg (interquartile range [IQR]=46-60) with a median pulmonary vascular resistance index (PVRi) of 9 WU*M-2 (IQR=8-22). All were acute pulmonary vasodilator testing non-responsive. All patients received targeted therapies. Three of five patients (60%) died despite an evidence-based approach to care. Of those who died, timing from the PoPH diagnosis to death ranged from three days to three years. Based upon our limited experience, PoPH is a disorder with significant mortality in childhood and challenges in treatment. Future research, focused on screening and early targeted treatment strategies, may alter the current dismal prognosis for these children.
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Details
- Title
- Portopulmonary hypertension in children: a rare but potentially lethal and under-recognized disease
- Creators
- Jennifer Tingo - Morgan Stanley Children's HospitalErika B. Rosenzweig - Morgan Stanley Children's HospitalSteven Lobritto - Morgan Stanley Children's HospitalUsha S. Krishnan - Morgan Stanley Children's Hospital
- Publication Details
- Pulmonary circulation, v 7(3), pp 712-718
- Publisher
- Sage
- Number of pages
- 7
- Resource Type
- Journal article
- Language
- English
- Academic Unit
- Pediatrics
- Web of Science ID
- WOS:000411119000018
- Scopus ID
- 2-s2.0-85032356434
- Other Identifier
- 991019168815404721
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InCites Highlights
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- Collaboration types
- Domestic collaboration
- Web of Science research areas
- Cardiac & Cardiovascular Systems
- Respiratory System