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Mystery still unresolved: Untouched "Blue heart" presenting at 40yrs of age
Sanjeev H Naganur1, Ashish Tiwari1, C R Pruthvi1
1Department of Cardiology, Post Graduate Institute of Medical Education and Research, Chandigarh, India.
Insights
Survival into adulthood with unrepaired dextro-transposition of great arteries (d-TGA) is rare. This report details an unusual case of a 40-year-old patient with d-TGA, presenting with stable symptoms and moderate pulmonary hypertension.
Area of Science:
- Cardiology
- Congenital Heart Disease
- Adult Congenital Heart Disease
Background:
- Dextro-transposition of the great arteries (d-TGA) is a severe cyanotic congenital heart disease.
- Surgical correction is typically required in infancy for survival.
- Survival into adulthood with unrepaired d-TGA is exceptionally rare.
Observation:
- This case presents a 40-year-old individual with unrepaired d-TGA.
- The patient exhibited moderately elevated pulmonary artery pressures.
- Symptoms were relatively stable despite the condition's severity.
Findings:
- The case challenges the typical lethal prognosis of unrepaired d-TGA.
- The presence of intracardiac shunts did not prevent late adult presentation.
- This highlights potential, yet unexplained, compensatory mechanisms in some d-TGA patients.
Implications:
- This case underscores the need for further research into long-term survival factors in unrepaired congenital heart disease.
- It may inform diagnostic and management strategies for adult congenital heart disease patients.
- Understanding such rare presentations can improve clinical awareness and patient care for complex cardiac conditions.
Abstract:
The modern-day surgical techniques and strategies have changed the outlook of patients with dextro-transposition of great arteries (d-TGA). The survival of an unrepaired d-TGA into late adulthood is difficult to explain. Even when large intracardiac shunts are present, it still remains a lethal cyanotic congenital heart disease if it is not surgically corrected soon after birth. Here, we report an extremely rare case of d-TGA presenting at 40 years of age, with moderately elevated pulmonary artery pressures and relatively stable symptoms.
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