Hypoplastic Left Heart Syndrome in a Neonate with Tracheoesophageal Fistula: An Anesthesiologist’s Nightmare!

To the Editor, Congenital esophageal atresia (EA) with or without tracheoesophageal fistula (TEF) occurs in approximately 1 in 2500 to 1 in 4000 live births.[1] Nearly 50% of these cases have associated congenital anomalies, of which cardiac defects are the most frequent. Hypoplastic left heart syndrome (HLHS), a rare and severe congenital heart defect with an incidence of 2 per 10,000 live births, may occasionally coexist with EA/TEF.[2] Few reports exist detailing anesthetic management in such cases, making this case a valuable contribution to the limited literature. We report the case of a preterm female neonate (34 weeks, 2 kg), antenatally diagnosed with HLHS, who presented with EA and distal TEF. Postnatal echocardiography confirmed hypoplastic left atrium, ventricle with a large Atrial and Ventricular Septal Defect. Dysmorphic features included a short neck, maxillary hypoplasia, low set large ears, and short palpebral fissures [Figures 1 and 2]. No additional congenital anomalies were identified. Following initial stabilization, with nasal continuous positive airway pressure, fluids, antibiotics, and other supportive care, she was planned for surgical repair of TEF followed by Norwood stage I palliation after taking informed written consent from parents. Anesthesia was induced with fentanyl, propofol, and atracurium; intubation was difficult and performed under CMAC guidance with size 3 uncuffed endotracheal tube with stylet (Cormack Lehane 2b). Intraoperatively, she experienced desaturation and hypotension, managed with oxygen titration, surgical retraction release, and vasopressors. Due to ongoing instability, the baby remained ventilated postoperatively. Despite intensive care, she succumbed to sepsis and cardiac compromise on postoperative day 2.Figure 1: Facial features of the neonate with short neck, maxillary hypoplasia, low-set, and big earsFigure 2: Facial features of the neonate with short eye apertureSaade and Setzer reported a term neonate with HLHS who underwent successful TEF repair followed by Norwood Stage I palliation 10 days later.[3] Despite surgical success, the patient suffered postoperative cardiac arrest, required ECMO, and ultimately did not survive. Similarly, Diaz et al. reviewed 25 cases of TEF/EA with congenital heart disease; among six duct-dependent lesions (like HLHS), only one infant survived following TEF repair, yielding a mortality rate of 83.3%.[4] Predictors of poor outcome in EA/TEF include low birth weight, major cardiac anomalies, and pulmonary infections.[5] These patients face higher rates of desaturation, difficult ventilation, inotropic support, and transfusion requirements. The presence of HLHS, combined with prematurity and low birth weight, significantly increases intraoperative instability and adverse outcomes. The cornerstone of anesthetic management in HLHS is achieving a pulmonary-to-systemic blood flow ratio (Qp: Qs) of ≤1 to maintain adequate tissue oxygenation. This is achieved by low FiO2 and permissive hypercapnia to induce pulmonary vasoconstriction, while maintaining systemic output through inotropes, afterload reduction, and blood transfusions.[3] In HLHS patients undergoing noncardiac surgery, elective repair is preferred after NICU stabilization. However, intraoperatively achieving this balance is particularly challenging due to unavoidable physiologic stressors like induction, intubation, bronchoscopy, one-lung ventilation and surgical manipulation. Nonetheless, coexisting HLHS and EA/TEF require preoperative optimization, parental counseling, preoperative optimization and consideration of palliative care in select cases. Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form, the legal guardian has given his consent for images and other clinical information to be reported in the journal. The guardian understands that names and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.