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  Table of Contents 
Year : 2021  |  Volume : 22  |  Issue : 2  |  Page : 193-195

Anesthetic management of an infant with uncorrected pentalogy of Fallot undergoing emergency exploratory laparotomy

1 Department of Anaesthesia, Atal Bihari Vajpayee Institute of Medical Sciences and Dr. Ram Manohar Lohia Hospital, New Delhi, India
2 Department of Cardiac Anaesthesia, Atal Bihari Vajpayee Institute of Medical Sciences and Dr. Ram Manohar Lohia Hospital, New Delhi, India
3 Department of Anaesthesiology and Intensive Care, Maulana Azad Medical College, New Delhi, India

Date of Submission27-Apr-2021
Date of Decision01-May-2021
Date of Acceptance03-May-2021
Date of Web Publication29-Sep-2021

Correspondence Address:
Dr. Nitin Choudhary
Flat No. 1601, Gardenia Gitanjali Apartments, Vasundhara Sector-18, Ghaziabad - 201 012, Uttar Pradesh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/TheIAForum.TheIAForum_78_21

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How to cite this article:
Choudhary N, Magoon R, Wadhawan S. Anesthetic management of an infant with uncorrected pentalogy of Fallot undergoing emergency exploratory laparotomy. Indian Anaesth Forum 2021;22:193-5

How to cite this URL:
Choudhary N, Magoon R, Wadhawan S. Anesthetic management of an infant with uncorrected pentalogy of Fallot undergoing emergency exploratory laparotomy. Indian Anaesth Forum [serial online] 2021 [cited 2023 May 30];22:193-5. Available from: http://www.theiaforum.org/text.asp?2021/22/2/193/326985


Anorectal malformations (ARMs) can be associated with congenital heart disease (CHD) necessitating staged palliative and/or corrective-procedures.[1] However, emergencies may necessitate a deviation from the surgical plan. Our case describes an emergency anesthetic management in setting of uncorrected pentalogy of Fallot (POF).

A 4-month-old 5 kg POF child on syrup propranolol 10 mg once-a-day (diagnosed POF during workup for 1st postnatal-day performance of colostomy for imperforate anus) presented with acute intestinal obstruction. Agitated and dehydrated, the vitals were temperature: 100°F, pulse: 134/min, blood pressure: 70/40 mmHg, respiratory rate: 30–34/min, and room-air oxygen saturation: 84% with intermittent cyanotic spells. There was a Grade I systolic murmur in pulmonary area with bilateral equal air entry. Investigations demonstrated polycythemia (Hb: 18.5 gm%) with leukocytosis (13,500/mm3) with mildly deranged renal function. Arterial blood gas (ABG) was pH: 7.29, pO2: 52 mmHg, pCO2: 28 mmHg, bicarbonate: 12 mmol/L, and base deficit: −10. Electrocardiogram showed right ventricular hypertrophy (RVH). Chest X-ray depicted a boot-shaped heart with oligemic lung fields. Echocardiography revealed atrial septal defect (ASD; ostium secundum), large subaortic ventricular septal defect (VSD) with predominantly right-to-left (R-L) shunt, aortic over-ride, RVH, severe pulmonary-valve stenosis with 90 mmHg peak valvular gradient, and a normal biventricular-function.

The child was calmed, fluid resuscitated (10 ml/kg), acidosis corrected with 1 mEq/Kg sodium bicarbonate, IV paracetamol 7.5 mg/kg, and knee-chest-positioning employed to terminate the tet spells. Subsequent to wheeling inside operation theater for an emergency exploratory laparotomy, standard ASA monitoring was applied. Following pre-oxygenation, modified rapid sequence induction was performed with fentanyl 1 μg/kg and ketamine 2 mg/kg intravenous (IV). Thereafter, succinylcholine 2 mg/kg IV was administered. The patient was intubated with 4 mm ID uncuffed endotracheal tube. The radial arterial pressure monitoring line and a 4.5 Fr triple-lumen central venous catheter were secured. The bladder was catheterized. In the lateral position, a caudal catheter was placed, checked, and fixed under ultrasound guidance and aseptic precautions.

Anesthesia was maintained with sevoflurane, oxygen and air, targeting 90%–95% oxygen saturation. Intraoperative tet spells (detected by end-tidal carbon dioxide decline) were managed with increased FiO2, femoral compression, and phenylephrine 20 μg IV. Inj. bupivacaine (0.125%) was administered in 2 ml aliquots via caudal catheter. Neuromuscular blockade was maintained with atracurium. Serial ABGs were performed. Paracetamol 7.5 mg/kg IV was administered 30 min before completion of the 1.5 h long surgery with an estimated 30 ml bloodloss. Neuromuscular blockade was reversed with neostigmine 50 μg/kgIV and glycopyrrolate 10 μg/kgIV. The trachea was extubated on the return of adequate tone and reflexes and the child was observed for 24 h in intensive care unit and discharged after uneventful 10 days.

ARM may be associated with CHD, vertebral defects, tracheoesophageal fistula, renal anomalies, and limb defects.[1] POF is a rare cyanotic CHD (3.7%) comprising of pentad of ASD, RVH, pulmonary stenosis, VSD, and aortic over-ride.[2] The key concern in POF is to prevent an augmented R-L shunt.[3] Herein, anesthesia is a balancing act between systemic vascular resistance (SVR) and pulmonary vascular resistance (PVR) while maintaining cardiac contractility and preload.[3],[4] Any PVR increase or SVR decrease would worsen the shunt. Hypoxia, hypercarbia, pain, acidosis, nitrous oxide, and positive end-expiratory pressure are the predisposing factors that must be avoided.[4] Ketamine helps in SVR maintenance, though one should be thoughtful regarding tachycardia.[3],[4] Inotropes and vasopressors should be available to correct hypotension. Given the possibility of paradoxical air embolism, intravenous tubings must be properly de-aired.[2],[3],[4]

The attending anesthesiologist must be vigilant to detect and treat perioperative tet spell. Fluid resuscitation, acidosis correction, multimodal analgesia, sedation, and parental accompaniment help in decreasing tet spell.[2],[4] Antibiotic prophylaxis against infective endocarditis should be administered as per the guidelines.[5]

A thorough understanding of the underlying pathophysiology with vigilant hemodynamic-monitoring forms the cornerstone of successful anesthetic management of POF.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Abadi SA, Abadi NA, Mashrabi O, Fatorachi H. Congenital hear anomalies in babies with imperforate anus and its motility. Res J Biol Sci 2008;3:922-24.  Back to cited text no. 1
Gabhane SK, Gangane NM, Sinha RT. Pentalogy of fallot and cardiac paraganglioma: A case report. Cases J 2009;2:9392.  Back to cited text no. 2
Ho YC, Boey SK, Varughese Mathews AM, See HG, Hwang NC. An unusual case of a parturient with uncorrected pentalogy of fallot presenting for elective cesarean section delivery of twins. Anesth Essays Res 2018;12:267-70.  Back to cited text no. 3
[PUBMED]  [Full text]  
Marulasiddappa V, Raghavavendra BS. Anesthesia for a rare case of uncorrected pentalogy of fallot undergoing craniotomy and drainage of brain abscess. J Clin Diagn Res 2015;9:D01-2.  Back to cited text no. 4
Thornhill MH, Dayer M, Lockhart PB, Prendergast B. Antibiotic prophylaxis of infective endocarditis. Curr Infect Dis Rep 2017;19:9.  Back to cited text no. 5


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