International Clinical and Medical Case Reports Journal (ISSN: 2832-5788) | Volume 4, Issue 11 | Case Report | Open Access
Abhimanyu Uppal*
Abhimanyu Uppal1*, Bhushan Shah2, Rambabu Sharma3, Ashok Garg4 1MD, DM Cardiology, PHCC, Jaipur, India
2MD, DNB Cardiology, AIIMS Bhopal, India
3MD Peditarics, JK Lon Hospital, Jaipur, India
4MD Medicine, PHCC, Jaipur, India
*Correspondence to: Abhimanyu Uppal
Fulltext PDFBackground: Pentalogy of Cantrell (PC) with ectopia cordis (EC) is rare and often fatal. Intracardiac anomalies, particularly secundum atrial septal defect (ASD), are common, occurring in ~80% of cases. However, percutaneous ASD closure prior to chest wall reconstruction has not been reported.
Case: A 14‑month‑old, 6.9‑kg girl with unrepaired PC‑EC, with history of recurrent respiratory infections, and failure to thrive had a large secundum ASD (~11–12 mm) with flail IVC rim. Given high surgical risk, transcatheter closure was attempted. The unique challenges included (i) poor echocardiographic visualization of defect and (ii) extreme cardiac mobility due to deficient sternum causing repeated device prolapse. Using a left‑atrial (LA) roof technique, a 14‑mm Lifetech ASD occlude device was successfully deployed a gentle wiggle maneuver assessed on bed-side echocardiography confirmed optimal device position before release.
Outcome: Three‑month follow‑up showed complete occlusion, no cardiac complications, weight gain (~4 kg), and resolution of infections.
Conclusion: Percutaneous ASD closure may serve as a bridge to definitive repair in selected PC‑EC patients. The case underscores the unique challenges of transcatheter ASD closure in setting of a deficient chest wall and highlights the importance of operator familiarity with alternative device deployment techniques.
Ectopia cordis; Pentalogy of Cantrell; Atrial septal defect; Transcatheter closure; Left atrial roof technique; Chest wall defect
Abhimanyu Uppal, Bhushan Shah, Rambabu Sharma, Ashok Garg. Transcatheter Atrial Septal Defect Closure in Unrepaired Pentalogy of Cantrell with Ectopia Cordis: First Reported Case. Int Clinc Med Case Rep Jour. 2025;4(11):1-10.