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Background <p>Congenitally corrected transposition of the great arteries (ccTGA) is an uncommon congenital heart defect involving both atrioventricular (AV) and ventriculoarterial (VA) discordance. This condition is further complicated by additional anomalies such as a hypoplastic pulmonary valve, ventricular septal defect (VSD), and dextroversion. These abnormalities may contribute to an increased risk of heart failure and cardiogenic shock, making early detection and appropriate intervention essential for better patient outcomes.</p> Case presentation <p>A 16-year-old female presented with progressive shortness of breath, cyanosis, recurrent episodes of syncope, and exertional palpitations. Upon admission, the patient exhibited severe hypoxia, tachycardia, and hypotension, consistent with cardiogenic shock. She was NYHA grade IV at the time of presentation.Imaging studies, including chest X-ray and echocardiography, GLS- strain imaging and cardiac MRI confirmed the diagnosis of ccTGA with AV and VA discordance, a left-sided aortic arch, subaortic VSD, a hypoplastic pulmonary valve, and left sided morphologic right ventricular systolic dysfunction. Blood investigations indicated polycythemia and signs of congestive hepatopathy.</p> Management and outcome <p>The patient was initially stabilized with inotropic support using norepinephrine (0.05–0.5 mcg/kg/min), along with diuretics and ACE inhibitors. Over the course of seven days, her condition improved significantly, allowing for the withdrawal of inotropic support. She was subsequently referred for surgical evaluation, with potential options including the double-switch procedure or VSD closure, depending on the extent of structural and functional abnormalities. Our patient underwent double switch procedure despite having RV dysfunction and improved significantly over a period of 3 months.</p> Conclusion <p>This case underscores the significance of prompt recognition and aggressive management in complex congenital heart conditions like ccTGA. Cases of symptomatic ccTGA in adolescence are exceptionally rare, highlighting the importance of a multidisciplinary approach in optimizing patient care and surgical planning.</p>

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Adolescent presentation of L-type ccTGA with pulmonary valve hypoplasia and RV dysfunction: from cardiogenic shock to surgical repair

  • 期刊论文

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  • 发表日期：

    2026年4月1日

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  • Shubham Jain,
  • Deepak Kumar,
  • Naween Kumar,
  • Pankaj Mohan Shrivastava

摘要

Background

Congenitally corrected transposition of the great arteries (ccTGA) is an uncommon congenital heart defect involving both atrioventricular (AV) and ventriculoarterial (VA) discordance. This condition is further complicated by additional anomalies such as a hypoplastic pulmonary valve, ventricular septal defect (VSD), and dextroversion. These abnormalities may contribute to an increased risk of heart failure and cardiogenic shock, making early detection and appropriate intervention essential for better patient outcomes.

Case presentation

A 16-year-old female presented with progressive shortness of breath, cyanosis, recurrent episodes of syncope, and exertional palpitations. Upon admission, the patient exhibited severe hypoxia, tachycardia, and hypotension, consistent with cardiogenic shock. She was NYHA grade IV at the time of presentation.Imaging studies, including chest X-ray and echocardiography, GLS- strain imaging and cardiac MRI confirmed the diagnosis of ccTGA with AV and VA discordance, a left-sided aortic arch, subaortic VSD, a hypoplastic pulmonary valve, and left sided morphologic right ventricular systolic dysfunction. Blood investigations indicated polycythemia and signs of congestive hepatopathy.

Management and outcome

The patient was initially stabilized with inotropic support using norepinephrine (0.05–0.5 mcg/kg/min), along with diuretics and ACE inhibitors. Over the course of seven days, her condition improved significantly, allowing for the withdrawal of inotropic support. She was subsequently referred for surgical evaluation, with potential options including the double-switch procedure or VSD closure, depending on the extent of structural and functional abnormalities. Our patient underwent double switch procedure despite having RV dysfunction and improved significantly over a period of 3 months.

Conclusion

This case underscores the significance of prompt recognition and aggressive management in complex congenital heart conditions like ccTGA. Cases of symptomatic ccTGA in adolescence are exceptionally rare, highlighting the importance of a multidisciplinary approach in optimizing patient care and surgical planning.