Factors Associated with Development of Post-Operative Pulmonary Hypertensive Crisis in Children with Congenital Heart Disease

Aims Pulmonary arterial hypertension is a severe complication in patients with congenital heart disease and poses a significant risk to women wishing to become pregnant. This study describes the clinical presentation, maternal outcomes and risk factors for the peripartum period in women with pulmonary arterial hypertension related to congenital heart disease (PAH-CHD). Methods All pregnant women with PAH-CHD who were admitted for delivery in a tertiary center between February 2011–September 2016 were included. Logistic regression analysis was used to identify predictors of the combined endpoint of maternal death, severe heart failure requiring treatment, or pulmonary hypertensive crisis. Results Ninety-three women (94 pregnancies) were included. Average age was 27.5 ± 4.4 years. Thirty (31.9%) patients had Eisenmenger syndrome, 51 (54.3%) had pulmonary arterial hypertension associated with systemic-to-pulmonary shunts, and 13 (13.8%) had pulmonary arterial hypertension with corrected congenital heart disease. Twenty-three (24.5%) women required admission for delivery within two days from presentation. Elective Cesarean section was performed in 95.7% of women, with intravertebral anesthesia in 93.6%. Fifty-one (54.2%) patients received pulmonary arterial hypertension therapies during pregnancy. Six (6.4%) women died, 33 (35.1%) developed heart failure and 10 (10.6%) had a pulmonary hypertensive crisis. Patients who met the combined endpoint ( n = 34, 36.2%) were more likely to have Eisenmenger syndrome or repaired defects ( p < 0.001). Other risk factors in the multivariate model included lower arterial blood oxygen saturation, higher brain natriuretic peptide, and pericardial effusion on echocardiography. Conclusion Maternal mortality and morbidity remain high in PAH-CHD patients, who should be counseled on the risks of pregnancy and managed in a tertiary multidisciplinary environment to improve prognosis.

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Aerosolized iloprost for postoperative pulmonary hypertensive crisis in children with congenital heart disease

International Journal of Cardiology ◽

10.1016/j.ijcard.2007.08.084 ◽

2008 ◽

Vol 129 (3) ◽

pp. 333-338 ◽

Cited By ~ 45

Author(s):

Alisa Limsuwan ◽

Suthep Wanitkul ◽

Anant Khosithset ◽

Sukasom Attanavanich ◽

Piya Samankatiwat

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Congenital Heart ◽

Hypertensive Crisis ◽

Pulmonary Hypertensive Crisis

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Pulmonary Arterial Hypertension In Adults With Congenital Heart Disease: General Overview of Disease Mechanisms

Advances in Pulmonary Hypertension ◽

10.21693/1933-088x-6.3.121 ◽

2007 ◽

Vol 6 (3) ◽

pp. 121-125 ◽

Cited By ~ 1

Author(s):

Ingram Schulze-Neick ◽

John E. Deanfield

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Pulmonary Arterial Hypertension ◽

Arterial Hypertension ◽

Congenital Heart ◽

Vascular System ◽

Hypertensive Crisis ◽

Multidisciplinary Care ◽

Structural Heart Disease ◽

Pulmonary Arterial

Adults with congenital heart disease (CHD) have become a rapidly expanding group of complex patients requiring multidisciplinary care in specialty centers by those trained in CHD. They represent one of the most challenging subgroups of patients with pulmonary arterial hypertension (PAH) due to the presence of structural heart disease with or without coexisting cyanosis and its complications. The primary focus of attention for these patients is the lungs, whose vascular system is affected by shunt flow, or is also congenitally malformed, or has been altered by surgical procedures. When PAH develops, it affects physical exercise tolerance, travel to high altitudes, pregnancy, operability, and anesthesia (myocardial failure due to pulmonary hypertensive crisis), and thus general morbidity and mortality in this special patient group.

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Clinical Factors Associated with Cerebral Metabolism in Term Neonates with Congenital Heart Disease

The Journal of Pediatrics ◽

10.1016/j.jpeds.2016.12.061 ◽

2017 ◽

Vol 183 ◽

pp. 67-73.e1 ◽

Cited By ~ 6

Author(s):

Anna Lonyai Harbison ◽

Jodie K. Votava-Smith ◽

Sylvia del Castillo ◽

S. Ram Kumar ◽

Vince Lee ◽

...

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Congenital Heart ◽

Cerebral Metabolism ◽

Clinical Factors ◽

Term Neonates ◽

Factors Associated

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Factors Associated with the Need for, and the Impact of, Extracorporeal Membrane Oxygenation in Children with Congenital Heart Disease during Admissions for Cardiac Surgery

Children ◽

10.3390/children4110101 ◽

2017 ◽

Vol 4 (11) ◽

pp. 101 ◽

Cited By ~ 2

Author(s):

Salvatore Aiello ◽

Rohit Loomba

Keyword(s):

Congenital Heart Disease ◽

Cardiac Surgery ◽

Heart Disease ◽

Extracorporeal Membrane Oxygenation ◽

Congenital Heart ◽

Factors Associated ◽

Membrane Oxygenation ◽

The Impact

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Factors Associated with Perceived Cognitive Problems in Children and Adolescents with Congenital Heart Disease

Journal of Clinical Psychology in Medical Settings ◽

10.1007/s10880-012-9326-z ◽

2012 ◽

Vol 20 (2) ◽

pp. 192-198 ◽

Cited By ~ 7

Author(s):

Christine A. Limbers ◽

Kara Emery ◽

Karen Uzark

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Children And Adolescents ◽

Congenital Heart ◽

Factors Associated ◽

Cognitive Problems

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A United Network for Organ Sharing analysis of heart transplantation in adults with congenital heart disease: Outcomes and factors associated with mortality and retransplantation

Yearbook of Cardiology ◽

10.1016/j.ycar.2011.04.010 ◽

2011 ◽

Vol 2011 ◽

pp. 168-169 ◽

Cited By ~ 3

Author(s):

V.H. Thourani

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Heart Transplantation ◽

Congenital Heart ◽

Factors Associated ◽

Disease Outcomes ◽

United Network

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Abstract 14215: Cognitive Impairment in Adult Congenital Heart Disease Survivors: A Pilot Study

Circulation ◽

10.1161/circ.142.suppl_3.14215 ◽

2020 ◽

Vol 142 (Suppl_3) ◽

Author(s):

Carla P Rodriguez Monserrate ◽

Rajeshwari Jakkam ◽

Emily Clay ◽

Kimberlee Gauvreau ◽

Michelle Z GURVITZ

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Cognitive Impairment ◽

Mild Cognitive Impairment ◽

Pilot Study ◽

General Population ◽

Ventricular Function ◽

Congenital Heart ◽

Education Level ◽

Factors Associated

Introduction: The most common comorbidities in children with congenital heart disease (CHD) are neurodevelopmental and psychosocial impairments, particularly in areas of executive function, memory, attention, and behavioral control. Limited studies in the adult CHD population suggest similar impairments exist and adults with CHD may be at increased risk for dementia. No studies have screened specifically for mild cognitive impairment and dementia in adult CHD patients. Methods: We performed a prospective cross-sectional study of adult CHD patients, ages 30-65 years, who were coming for routine clinic visits. We administered the Mini-Mental State Exam (MMSE) and scores were compared with population norms adjusted by age and education level. We also evaluated the association of MMSE scores with CHD complexity, demographic and clinical risk factors. Results: A total of 125 patients were recruited (55% male). The median age was 40 years (range 30-65). Almost all participants (97%) had a high school degree and 75% had some college education or advanced degrees. The majority of patients (94%) had moderate or complex CHD. Adjusting for age and education, CHD participants scored significantly lower than the general population (median 1 point lower, p=0.001). The greatest impairments occurred in recall and orientation. Factors associated with lower scores included decreased systemic ventricular function (p=0.028) and having ≥2 cardiac catheterizations (p=0.006). Five percent of the total cohort met the general threshold for mild cognitive impairment (MMSE<24). Clinical factors associated with this degree of cognitive impairment were duration of cyanosis (p=0.005) and decreased systemic ventricular function (p=0.003). Conclusions: Our pilot study showed that, when adjusted for age and education level, adult CHD patients had significantly lower MMSE scores than the general population, with 5% meeting criteria for mild cognitive impairment. These findings suggest that subtle and early neurodevelopmental changes are present in the adult CHD population. Further studies are needed to investigate those changes and evaluate potential disease modifying therapies that might influence long-term outcomes in the adult CHD population.

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