1-month-old baby with congenital LV apical aneurysm treated at Paras Hospitals Gurugram

Gurugram: Paras Health, a leading healthcare provider in Gurugram, recently achieved a groundbreaking medical milestone by diagnosing and successfully treating a rare congenital heart defect in a 1-month-old infant, making it the youngest case of its kind in the world. 

The case involved a congenital LV apical aneurysm, a condition where the apical part of the left ventricle of the heart is replaced by fibrous tissue, making it thin and weak, and prone to spontaneous rupture. The condition is exceptionally rare, with only 809 cases reported worldwide since 1816.

Congenital LV apical aneurysm presents significant structural and functional challenges in the heart, as the normal muscular tissue is replaced by fibrous tissue. This condition can remain undiagnosed for years after birth and poses a serious risk of sudden cardiac events, such as sudden infantile death syndrome (SIDS), especially during periods of stress such as crying or physical activity.

Reflecting on this achievement, Dr Mahesh Wadhwani, Chief & HOD - Paediatric & Adult Cardiothoracic Vascular Surgery at Paras Health, remarked, "The successful treatment of this intricate case in such a young child represents a significant milestone not only for Paras Health but also for the global medical community.

This indicates the excellence of surgical work that we are currently performing and a level of maturity in functioning of our operation theatre and ICU to handle such complex cases well. It epitomizes the relentless efforts and collaboration of our entire team in overcoming complex hurdles to achieve a successful outcome, showcasing the holistic approach from awareness to diagnostics, screening, treatment, and post-treatment care.”

Commenting on the diagnosis, Dr Deepak Thakur, Senior Consultant – Paediatric & Fetal Cardiology, Paras Hospitals, Gurugram, “Congenital heart defects affect the structure of the heart and are typically detected either at birth or before. In this extraordinary case, the condition was diagnosed during fetal echocardiography at 26 weeks of pregnancy.

The fetal echocardiography played an important role to help diagnose such rare anomaly and further helped in planning the course of treatment. After diagnosis, the child was closely monitored with bi-weekly follow-ups until delivery to ensure that the size of the aneurysm did not increase. Following birth, an MRI was performed to confirm the diagnosis, which corroborated with Echocardiography findings and revealed thinned out Lv apical part > 1/4th of Lv volume and non-functional part. The parents were advised for close follow up on medical management; however, after a month, when the size of the aneurysm increased and the child started experiencing breathing difficulties, it was decided to perform the surgery.”

The surgery was performed when the baby was 1 month weighing 3.4 kg – the doctors planned strict cardiac monitoring and let the baby grow as old as possible before operating allowing the heart to grow muscles. The doctors at Paras Health collectively decided on the best course of action and meticulously planned the surgery, involving clinical Pediatric cardiac team of Pediatric cardiologist, Cardiac surgeon, perfusionists and cardiac anaesthetists.

Dr Shyamveer Singh Khangarot, Consultant -Congenital (Neonatal & Pediatric) Cardiac Surgeon, Paras Health, Gurugram, emphasized the unique challenges posed by treating this rare condition in an infant, stating, "Neonatal and infant cardiac surgeries are inherently complex, however the rarity and complexity of this disease made the task even more challenging.

The involvement of the left side of the heart added another layer of complexity. The surgical procedure performed, known as Left Ventricular Endoaneurysmorraphy, required meticulous planning due to the infant's small heart size. Every millimetre of tissue in the infant's heart was critical, and we had to carefully consider not only the immediate outcome but also the long-term growth potential.

After 6 hours of the surgery, the aneurysm was repaired with composite patch of polyester and pericardium with another layer of epicardium. Even before the surgery, contingency preparation was done to support baby on ECMO if required in the form of integrated ECMO / CPB circuit, given the uncharted nature of post-operative course due to rarity and complexity of procedure.“

Highlighting the importance of post treatment care, Dr Alok Sahoo (Cardiac Anesthetist), Paras Health, Gurugram said, “During the post-operative period, we maintained constant vigilance over the baby, ensuring optimal care of hemodynamics, respiratory function, and nutrition. The baby required ventilation for 36 hours post-operation, after which the child was discharged within a week.

The long-term implications in such cases could include cardiac dysfunction and arrhythmias necessitating regular follow-ups including echo and hemodynamic monitoring. For future care, it's imperative for parents to provide attentive care and ensure regular follow-ups, proper feeding, and adequate nutrition to support the baby's recovery and long-term health. Furthermore, given the infant's small size, meticulous nursing care is essential, including gentle handling of the baby.

Congenital heart diseases commonly affect infants and children, with approximately 10 out of every 100 children impacted by these conditions. The case study exemplifies Paras Health's commitment to providing advanced tertiary care, ensuring compassion, accessibility, affordability, and quality in all healthcare services offered.