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Published on: February 11, 2017
Left-to-Right Shunt with Congenital Heart Disease: Single Center Experience
Ayhan Cevik1, Rana Olgunturk, Serdar Kula
1Gazi University Medical Faculty Hospital, Department of Pediatric Cardiology, 06560 Ankara, Turkey.
Insights
Pulmonary arterial hypertension (PAH) affects 12.3% of congenital heart disease (CHD) patients with large left-to-right shunts. Perimembranous ventricular septal defects pose the highest risk, but age and gender do not correlate with PAH development.
Area of Science:
- Cardiology
- Pediatric Cardiology
- Pulmonary Hypertension
Background:
- Congenital heart disease (CHD) with left-to-right shunts can lead to pulmonary hypertension.
- Understanding the frequency and risk factors for pulmonary arterial hypertension (PAH) in this population is crucial for management.
Purpose of the Study:
- To determine the incidence of PAH in patients with CHD and isolated large left-to-right shunts.
- To identify factors contributing to the development of PAH in this cohort.
Main Methods:
- Comparison of pressure measurements and Fick's principle calculations across three patient groups: PAH, hyperkinetic pulmonary hypertension (HPH), and neither.
- Statistical analysis including t-tests and multiple linear regression.
Main Results:
- PAH was diagnosed in 12.3% of patients, HPH in 14.4%, and 73.1% had neither.
- Perimembranous ventricular septal defect was associated with the highest risk of PAH.
- Significant differences in left atrial pressure and mean pulmonary arterial pressure were observed between groups.
Conclusions:
- The study did not establish a correlation between the duration of high pulmonary flow and increased pulmonary vascular resistance or PAH development in isolated left-to-right shunts within CHD.
- Further research may be needed to elucidate the complex pathophysiology of PAH in CHD.
Abstract:
Objective. The objective of this study was to determine the frequency of pulmonary arterial hypertension (PAH) in congenital heart disease (CHD) with an isolated, large left-to-right shunt and to indicate the factors in the development of PAH. Methods. The pressure measurements in the cardiac chambers and the calculations based on the Fick's principle were compared among 3 separate groups of patients, respectively, with PAH, with hyperkinetic pulmonary hypertension (HPH), and with neither PAH nor HPH. Results. PAH was diagnosed in 30 (12.3%) patients, HPH in 35 (14.4%), while 177 (73.1%) were free of either. The highest risk for the development of PAH was found in the presence of perimembranous ventricular septal defect. A statistically significant difference was seen among these groups as to their left atrial pressure (p = 0.005) and the mean pulmonary arterial pressure (PAPmean; p < 0.001). While a correlation was present between RpI on one hand and age on the other (p = 0.014), a multiple linear regression could not evidence any correlation among age (p = 0.321), gender (p = 0.929). Conclusion. Our findings do not allow establishing a correlation between the duration of the high pulmonary flow and pulmonary vascular resistance increase or PAH development in isolated left-to-right shunts with congenital heart diseases.
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