Pulmonary hypertension in adults with congenital heart disease. Clinical phenotypes and outcomes in the advanced pulmonary vasodilator era

OBJECTIVE:To determine the frequence and profile of congenital heart defects in Down syndrome patients referred to a pediatric cardiologic center, considering the age of referral, gender, type of heart disease diagnosed by transthoracic echocardiography and its association with pulmonary hypertension at the initial diagnosis.METHODS:Cross-sectional study with retrospective data collection of 138 patients with Down syndrome from a total of 17,873 records. Descriptive analysis of the data was performed, using Epi-Info version 7.RESULTS: Among the 138 patients with Down syndrome, females prevailed (56.1%) and 112 (81.2%) were diagnosed with congenital heart disease. The most common lesion was ostium secundum atrial septal defect, present in 51.8%, followed by atrioventricular septal defect, in 46.4%. Ventricular septal defects were present in 27.7%, while tetralogy of Fallot represented 6.3% of the cases. Other cardiac malformations corresponded to 12.5%. Pulmonary hypertension was associated with 37.5% of the heart diseases. Only 35.5% of the patients were referred before six months of age.CONCLUSIONS: The low percentage of referral until six months of age highlights the need for a better tracking of patients with Down syndrome in the context of congenital heart disease, due to the high frequency and progression of pulmonary hypertension.

Download Full-text

Dilutional And Modified Ultrafiltration Reduces Pulmonary Hypertension After Operations For Congenital Heart Disease: A Prospective Randomized Study

Journal of Thoracic and Cardiovascular Surgery ◽

10.1016/s0022-5223(98)70313-7 ◽

1998 ◽

Vol 115 (3) ◽

pp. 517-527 ◽

Cited By ~ 59

Author(s):

Ko Bando ◽

Palaniswamy Vijay ◽

Mark W. Turrentine ◽

Thomas G. Sharp ◽

Lynda J. Means ◽

...

Keyword(s):

Congenital Heart Disease ◽

Pulmonary Hypertension ◽

Heart Disease ◽

Congenital Heart ◽

Randomized Study ◽

Prospective Randomized Study

Download Full-text

Quantitative Evaluation of Right Ventricle Function by Transthoracic Echocardiography in Childhood Congenital Heart Disease Patients with Pulmonary Hypertension

Echocardiography ◽

10.1111/j.1540-8175.2012.01697.x ◽

2012 ◽

Vol 29 (7) ◽

pp. 840-848 ◽

Cited By ~ 8

Author(s):

Ayhan Cevik ◽

Serdar Kula ◽

Rana Olgunturk ◽

F. Sedef Tunaoglu ◽

A. Deniz Oguz ◽

...

Keyword(s):

Congenital Heart Disease ◽

Pulmonary Hypertension ◽

Heart Disease ◽

Right Ventricle ◽

Quantitative Evaluation ◽

Transthoracic Echocardiography ◽

Congenital Heart ◽

Right Ventricle Function ◽

Ventricle Function

Download Full-text

Nutritional assessment and associated factors in children with congenital heart disease in Ethiopia.

10.1101/2021.04.12.21255315 ◽

2021 ◽

Author(s):

Temesgen Tsega Desta

Keyword(s):

Congenital Heart Disease ◽

Pulmonary Hypertension ◽

Heart Disease ◽

Associated Factors ◽

Congenital Heart ◽

Nutritional Assessment ◽

Failure To Thrive ◽

Cross Sectional ◽

Care Givers ◽

Increased Risk

ABSTRACT Infants and children with congenital heart disease exhibit a range of delays in weight gain and growth. In some instances, the delay can be relatively mild, whereas in other cases, cause the failure to thrive. OBJECTIVES To determine the nutritional status and associated factors of pediatric patients with congenital heart disease. MATERIAL AND METHODS A cross sectional analytical study was done over a period of 6months (Feb to Jul 2020). A total of 228 subjects with congenital heart disease that come to the cardiac center during the study period where included until the calculated sample size was attained. Data was collected from patient card and care givers of the children included in the study after obtaining their informed consent using data inquiry sheet. RESULTS A total of 228 children from age 3month to 17yrs. Most of the subjects had acyanotic heart disease accounting for 87.7%. The overall prevalence of wasting, underweight and stunting were 41.3%, 49.1% and 43% respectively. Among this children with congenital heart disease those with PAH were found more likely have wasting compared to those without PAH with an odds of 1.9 (95% CI: 1.0-3.4) and also greater chance of being stunted with an odds of 1.9 (95% CI: 1.0-3.4). children above 5years of age were 2.3 times more likely to be underweight. CONCLUSION Malnutrition is a major problem of patients with CHD. Pulmonary hypertension and older age are associated with increased risk of undernutrition. KEYWORDS: Acyanotic, cyanotic, Pulmonary hypertension, underweight, wasting and stunting.

Download Full-text

Pulmonary vasodilator therapy is associated with greater survival in Eisenmenger syndrome

Heart ◽

10.1136/heartjnl-2017-311876 ◽

2017 ◽

Vol 104 (9) ◽

pp. 732-737 ◽

Cited By ~ 8

Author(s):

Clare Arnott ◽

Geoff Strange ◽

Andrew Bullock ◽

Adrienne C Kirby ◽

Clare O’Donnell ◽

...

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Congenital Heart ◽

Adult Congenital Heart Disease ◽

Single Agent ◽

Eisenmenger Syndrome ◽

Vasodilator Therapy ◽

Risk Of Death ◽

Complex Anatomy ◽

Pulmonary Vasodilator

ObjectiveEisenmenger syndrome (ES) is a severe form of pulmonary hypertension in adults with congenital heart disease (CHD) and has a poor prognosis. We aimed to understand factors associated with survival in ES and particularly to assess the potential benefits of advanced pulmonary vasodilator therapy (AT).MethodsFrom January 2004, when AT became generally available for patients with ES, we followed 253 ES adults from 12 adult congenital heart disease centres across Australia and New Zealand. Demographic, medical and outcome data were collected and analysed prospectively and retrospectively.ResultsThe patients with ES were predominantly female (60%), aged 31 (SD 12) years. At diagnosis of ES, 64% were WHO functional class ≥3. The most common underlying lesion was ventricular septal defect (33%) with 21% having ‘complex’ anatomy. Over a median follow-up time of 9.1 years, the majority (72%) had been prescribed at least one AT (49% single agent), mostly bosentan (66%, 168 patients). The mean time on AT was 6 (SD 3.6) years. Those on AT were more functionally impaired at presentation (69% WHO ≥3 vs 51%, p=0.007) and more likely to have been prescribed anticoagulation (47% vs 27%, p=0.003). The risk of death/transplant was 4.8 %/year in AT exposed versus 8.4% in those never exposed. On multivariable analysis, exposure to AT was independently associated with greater survival (survival HR 2.27, 95% CI 1.49 to 3.45; p<0.001). WHO ≥3 at presentation was associated with a worse prognosis (mortality HR 1.82, 95% CI 1.19 to 2.78; p=0.006).ConclusionTreatment with AT was independently associated with greater survival in patients with ES, even though they were comparatively sicker prior to treatment.

Download Full-text