Pulmonary Hypertension Associated With Congenital Heart Disease

Adatia, Ian; Kothari, Shyam Sunder; Feinstein, Jeffrey A.

doi:10.1378/chest.09-2861

Cited by 97 publications

(82 citation statements)

References 104 publications

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“…Worldwide, because of inequality of access to medical care, only some patients (2%-15%) undergo reparative surgery. 24 A large outcome study in PAH-CHD showed a prevalence of PAH in CHD of 5.8% and its presence increased all-cause mortality and cardiac complications, including heart failure and arrhythmias, more than 2-fold and 3-fold, respectively. 36 In addition, the presence of PAH increased hospital days and intensive care unit days by more than 3-fold when compared with patients with CHD without PAH.…”

Section: Epidemiologymentioning

confidence: 99%

“…23 The reason for including CHDs in group 1 is because its histology and endothelial cell abnormalities are indistinguishable from other causes of PAH. 24 Classically, all congenital conditions are grouped together when considered as a cause of PAH. However, it is important to identify the underlying congenital defect, because this has important prognostic implications.…”

Section: Definition and Classificationmentioning

confidence: 99%

“…[33][34][35] Worldwide, it is estimated that 3.2 million children are at risk for the development of PAH-CHD. 24 Most of them do not develop Eisenmenger syndrome, particularly if cardiac repair occurs within the first 2 years of life. Worldwide, because of inequality of access to medical care, only some patients (2%-15%) undergo reparative surgery.…”

Section: Epidemiologymentioning

confidence: 99%

“…24 It is recommended that children with large left-to-right shunts or increased PVR undergo operative closure of the defect in the first 12 to 18 months of life to prevent development of Eisenmenger syndrome. 158 In children younger than 2 years, pulmonary vascular remodeling is usually reversible after the repair of the heart defect.…”

Section: Preventionmentioning

confidence: 99%

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Congenital Heart Disease and Pulmonary Hypertension

Gupta

Tonelli

Krasuski

2012

Heart Failure Clinics

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Section: Epidemiologymentioning

confidence: 99%

Section: Definition and Classificationmentioning

confidence: 99%

Section: Epidemiologymentioning

confidence: 99%

Section: Preventionmentioning

confidence: 99%

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Congenital Heart Disease and Pulmonary Hypertension

Gupta

Tonelli

Krasuski

2012

Heart Failure Clinics

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“…This condition activated the sympathetic nervous system and the renin-angiotensin-aldosteron system (Giardini, et al, 2003, Madriago andSilberbach, 2010). Chronic adrenergic stimulation leads to worsening heart failure by inducing ventricle and vascularity remodelling (Cohn, et al, 2000;Marin, 2000;Roig, 2006;Adatia, et al, 2010).…”

mentioning

confidence: 99%

The Improvement Chest X-Ray After Carvedilol Theraphy in Heart Failure Due to Left to Right Shunt.

Rahman¹,

Zuraedah²,

Hidayat³

et al. 2017

IJAR

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heart failure, left to right shunt congenital heart defect, carvedilol, cardio-thoracic ratio.Background: Heart failure due to left to right shunt congenital heart defect will increase pulmonary blood flow and cause volume overload. This condition activated the sympathetic nervous system and the reninangiotensin-aldosteron system. Conventional therapy has not blocked the sympathetic system yet. Carvedilol, a novel non selective β-blocker, reduced mortality and hospitalization in adults with heart failure. Limited information was available about its use in children. Objective: To evaluate the effect of carvedilol on chest x-ray in children with heart failure due to left to right shunt congenital heart defect. Methods: A randomized, double-blind, placebo-controlled study was done. In addition to conventional therapy, patients were assigned to receive placebo or carvedilol. Carvedilol was initiated at a dose of 0.05 mg/kg/day, with a target dose of 0.2 mg/kg/day. Chest x-ray was done before-after treatment and evaluated for cardio-thoracic ratio and pulmonary vascularity. The data was analyzed using independent sample t-test and Chi-square test, with confidence interval 95%. Results: Of 30 patients, 15 in each group. The mean age was 57.6(SD 43.57) months, 19(63.3%) were boys. There were 21(70%) children with VSD and 9(30%) children with PDA. Compared to control group, children in the carvedilol group had a significant decrease of cardiothoracic ratio (-2.94±2.34% versus -0.48±3.19%, p=0.023, CI 95%:-4.556 to-0.360). However, there was no significant change of pulmonary vascularity(p=0.153). Conclusion: Carvedilol decreased the cardio-thoracic ratio on chest xray, but did not improve the pulmonary vascularity in children with heart failure due to left to right shunt Congenital heart defect.

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Iloprost for children with pulmonary hypertension after surgery to correct congenital heart disease

Zhu

Liu

et al. 2014

Pediatric Pulmonology

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Congenital heart disease (CHD) can cause pulmonary hypertension (PH) in children, and surgery to correct CHD may be complicated by postoperative pulmonary hypertensive crises (PHC). Clinical data regarding the use of inhaled iloprost to treat children with PH are scarce. Our aim was to determine the efficacy and safety of iloprost in children with PH following surgery to correct CHD. This was a randomized, placebo-controlled study of 22 children (median age 7 months) undergoing surgery to achieve biventricular repair. The combined clinical endpoint was a decrease of more than 20% in the ratio of systolic pulmonary arterial pressure to systolic arterial pressure or pulmonary resistance to systemic resistance, with no PHC or death. Patients were randomized to receive low-dose iloprost (30 ng/kg/min), high-dose iloprost (50 ng/kg/min), or placebo, for 10 min every 2 hr in the first 48 hr after surgery. PHC were experienced by two patients who received placebo and one patient treated with high-dose iloprost. The combined clinical endpoint was reached by six patients administered low-dose iloprost (P = 0.005) and four administered high-dose iloprost (P = 0.077), compared with none in the placebo group. Patients treated with iloprost showed a significant reduction from baseline in mean pulmonary vascular resistance index (-2.2 Wood units, P < 0.05), whereas patients who received placebo showed no significant change. This study supports the use of iloprost to treat children with PH following surgery to correct CHD.

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Pulmonary Hypertension Associated With Congenital Heart Disease

Cited by 97 publications

References 104 publications

Congenital Heart Disease and Pulmonary Hypertension

Congenital Heart Disease and Pulmonary Hypertension

The Improvement Chest X-Ray After Carvedilol Theraphy in Heart Failure Due to Left to Right Shunt.

Iloprost for children with pulmonary hypertension after surgery to correct congenital heart disease

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