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Published on: May 11, 2015
Prevalence of pulmonary hypertension in children with Prader-Willi Syndrome
Shirleen Kohn1, Mari Evans2, Reem Itani3
1Children's Hospital Los Angeles, Division of Pediatric Pulmonology and Sleep Medicine, 560 Sunset Blvd. MS 83, Los Angeles, CA, 90027, USA; Keck School of Medicine, University of Southern California, 1975 Zonal Avenue, Keith Administration 200, Los Angeles, CA, 90089-9023, USA.
Insights
Pulmonary hypertension (PH) affects 27% of children with Prader-Willi Syndrome (PWS) and sleep-related breathing disorders (SRBD), especially those with hypoventilation. Early PH screening is recommended for PWS patients with SRBD.
Area of Science:
- Pediatric Cardiology
- Pulmonology
- Genetics
Background:
- Prader-Willi Syndrome (PWS) is linked to obesity and sleep-related breathing disorders (SRBD).
- Pulmonary hypertension (PH) is common in children with SRBD.
Purpose of the Study:
- To determine PH prevalence in children with PWS and SRBD.
- To assess the association between PH, OSA severity, and obesity in PWS patients.
Main Methods:
- Retrospective study of 37 PWS patients at Children's Hospital Los Angeles.
- Collected data included demographics, BMI, echocardiograms, cardiac catheterization, and polysomnography (PSG).
Main Results:
- 27% of PWS patients with SRBD met PH criteria.
- PH was associated with obesity and SRBD (average OAHI 21.9).
- Hypoventilation showed a significant association with PH (p=0.003).
Conclusions:
- PH is prevalent in children with PWS, particularly those with hypoventilation.
- PH diagnosis occurred in later childhood, post-SRBD diagnosis.
- Routine PH screening is advised for PWS patients, especially with hypoventilation.
Rationale:
Prader-Willi Syndrome (PWS) is associated with obesity and sleep-related breathing disorders (SRBD). Pulmonary hypertension (PH) is prevalent in children with SRBD. We sought to determine the PH prevalence in children with PWS and SRBDs and determine the association of PH with OSA severity and obesity.
Methods:
A retrospective study of patients with PWS followed at Children's Hospital Los Angeles was performed. Data collected included demographics, body mass index (BMI), echocardiogram, cardiac catheterization (if present), and polysomnography (PSG) results.
Results:
37 patients with PWS had technically acceptable echocardiogram and PSG or chronic respiratory failure diagnosis; ten (27%) met criteria for PH. All patients with PH were obese and had SRBD with average OAHI of 21.9 ± 20 events/hour. Five patients had hypoventilation. The average age of first echocardiogram was 9.8 ± 2.8 years. The average age at PH diagnosis was 11.1 ± 3.9 years. The average TR jet was 2.8 ± 0.3 m/s. Four patients had septal flattening. One had cardiac catheterization with mean PA pressure 29 mmHg, PVR 3.7wu x m2, and RV systolic pressure 42 mmHg. Hypoventilation was associated with pulmonary hypertension (p = 0.003). There was no detectable association between presence of OSA, sleep related hypoxemia, elevated BMI or growth hormone therapy and the presence of PH.
Conclusions:
PH is relatively prevalent in children with PWS, particularly in those with hypoventilation. PH was identified in later childhood, after they had a diagnosis of SRBD. Our findings advocate for earlier and routine screening of PH particularly in those with hypoventilation.
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