Year : 2011  |  Volume : 14  |  Issue : 1  |  Page : 62--65

Perioperative management of a neonate presenting with midgut volvulus and obstructed infracardiac total anomalous pulmonary venous connection

Nilesh M Juvekar1, Sharmila S Deshpande1, Usha Pratap2, Ranjit R Jagtap3, Shreeprasad P Patankar4,  
1 Department of Anaesthesia and Critical Care, Deenanath Mangeshkar Hospital and Research Centre, Pune, India
2 Department of Cardiology, Deenanath Mangeshkar Hospital and Research Centre, Pune, India
3 Department of Cardiovascular and Thoracic Surgery, Deenanath Mangeshkar Hospital and Research Centre, Pune, India
4 Department of Paediatric Surgery, Deenanath Mangeshkar Hospital and Research Centre, Pune, India

Correspondence Address:
Nilesh M Juvekar
A 10 / 1004 źDQ╗KarishmaźDQ╗ Kothrud, Pune - 411 038

How to cite this article:
Juvekar NM, Deshpande SS, Pratap U, Jagtap RR, Patankar SP. Perioperative management of a neonate presenting with midgut volvulus and obstructed infracardiac total anomalous pulmonary venous connection.Ann Card Anaesth 2011;14:62-65

How to cite this URL:
Juvekar NM, Deshpande SS, Pratap U, Jagtap RR, Patankar SP. Perioperative management of a neonate presenting with midgut volvulus and obstructed infracardiac total anomalous pulmonary venous connection. Ann Card Anaesth [serial online] 2011 [cited 2021 Nov 29 ];14:62-65
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Full Text

The Editor,

Congenital heart diseases (CHDs) are common congenital birth defects, with the incidence varying from 4/1000 to 50/1000 live births. [1] Abnormalities of the gastrointestinal, musculoskeletal and urinary systems are frequently seen associated with CHD. [2] The incidence of intestinal malrotation (IM) is 1:500 to 1:3520 live births, [3],[4] occurring in 2.8-4.1% of all patients with CHD. [5] Volvulus, a rotation of the intestine along its mesenteric stalk, occurs in 60-70% of the neonates diagnosed with IM, in which strangulation is seen in about 15% of the patients. [6] IM with volvulus is a neonatal surgical emergency. Delay in surgery can negatively influence the outcome due to the development of intestinal necrosis, septicemia and short bowel syndrome. [7]

Total anomalous pulmonary venous connection (TAPVC) is a congenital malformation in which the pulmonary veins drain into the right atrium either directly or indirectly. The route to the heart may have areas of obstruction, causing hemodynamic compromise, which is more common with the infracardiac type. Both volvulus and obstructed infracardiac TAPVC need immediate surgical attention. The fluid and electrolyte imbalance caused by the volvulus can aggravate the preexisting fragile hemodynamic state of a child with infracardiac TAPVC and vice versa. Staged repair of the volvulus followed by TAPVC poses a risk of severe hemodynamic compromise in the perioperative period of surgery for volvulus endangering life, making combined surgery a preferred option.

A staged procedure can be considered only if the IM is not associated with the volvulus, in which obstructed TAPVC should be operated first.

A 17-day-old female child weighing 2.7 kg with a gestational age of 38 weeks was admitted to the hospital with the provisional diagnosis of volvulus as per the abdominal ultrasound scan.

Physical examination of the child revealed tachypnoea, grunting and retraction of the chest wall, heart rate of 153/min, systolic blood pressure of 60 mmHg with inotropic support of Milrinone 0.5 μ/kg/min and oxygen saturation of 85% with 2L O 2 /min. Echocardiography revealed obstructed infracardiac TAPVC with a 27 mm Hg gradient across the descending vein draining into the liver, severe pulmonary hypertension, small atrial septal defect (ASD), no ventricular septal defect, dilated right atrium, dilated right ventricle, small left atrium and left superior vena cava. Arterial blood gas (ABG) was normal and venous blood saturation was 57%. Serum K + was 2.6 mEq/L, which was corrected slowly by maintenance solution (10% dextrose 100 ml + 1 cc concentrated Ringer lactate + 1 cc KCl ) at 12.5 ml/h.

The chest X-ray revealed signs of pulmonary venous obstruction and normal cardiac size [Figure 1]. Barium upper gastrointestinal (GI) series was performed to reconfirm the diagnosis [Figure 2]. The child was scheduled for emergency repair of the infracardiac obstructed TAPVC and midgut malrotation with clinical diagnosis of volvulus.{Figure 1}{Figure 2}

General anesthesia was induced with intravenous ketamine, midazolam, vecuronium and inhalation of isoflurane with oxygen. The trachea was intubated with a 3.0 mm endotracheal tube. General anesthesia was maintained with oxygen, air, isoflurane and incremental doses of fentanyl and vecuronium as needed. The right internal jugular vein (IJV) was cannulated with a 4.5 Fr triple-lumen 6 cm line and the right femoral artery was cannulated with a 22G cannula.

Cardiopulmonary bypass (CPB) was instituted and repair of the TAPVC was performed under total circulatory arrest (TCA) at 20º (TCA time: 24 min). The CPB was weaned off and a nasopharyngeal temperature of 36ºC and skin temperature of 30ºC, heart rate of 150/min, blood pressure of 59/34 (42) mmHg, pulmonary artery pressure (PAP) of 30/18 (23) mmHg and central venous pressure (CVP) of 13 mmHg were recorded. Discontinuation of CPB was assisted with inotropic support of dopamine 10 μg/kg/min, milrinone 0.7 μg/kg/min, adrenaline 0.1 μ/kg/min and inhaled nitric oxide (iNO) at 20 ppm. (CPB time: 110/min, aortic cross clamp time: 28 min.) Conventional ultrafiltration (CUF) and modified ultrafiltration (MUF) were performed on the CPB. Hemodynamics improved after MUF, with PAP decreasing to less than half of the systemic pressure (HR 142/min, BP 93/57 (71) mmHg, PAP 34/17 (25) mmHg and CVP 11 mmHg). Heparin was reversed with protamine. The chest was kept open and sealed with an antimicrobial drape.

The abdomen was opened by a transverse supraumbilical incision. Midgut volvulus with clockwise 360º rotation was seen and derotation was done. Bowel vascularity appeared satisfactory after derotation. Ladd's procedure was performed and the abdomen was closed in layers.

Oral sildenafil 0.1 mg/kg 6-hourly was started immediately postoperatively and this was gradually increased to 0.5 mg/kg 6-hourly as per the institutional protocol. The patient was transferred with the chest open to the intensive care unit. Thirty-six hours later, the chest was closed. On the postoperative day 2, Furosemide infusion 1 mg/kg/h and Aminophylline infusion 0.8 mg/kg/h were started to ensure good urine output with the aim of negative fluid balance. Peritoneal dialysis was avoided in view of abdominal surgery. Twenty-four hours after chest closure, weaning of inotropes was commenced. Oral phenoxybenzamine was started on day 5 at 0.1 mg/kg/dose 8-hourly and increased at every dose by 0.1 mg to a dose of 0.3 mg/kg/dose 8-hourly to help wean off iNO and to avoid the occurrence of pulmonary hypertensive crisis after ceasing iNO. Ventilation was slowly weaned and the child was extubated on day 7 and continuous positive airway pressure (CPAP) with iNO 10 ppm was given through nasal prongs. The child required insertion of the right intercostal drain on day 11 for relief of respiratory distress due to pleural effusion. The patient was ventilated for 3 days during this episode. The child was re-extubated on day 14, and received additional intravenous antibiotics as per the blood culture and sensitivity report for 14 days and was discharged on day 26. Predischarge echocardiography and barium upper GI series were normal.

Tsvetomir Loukanov et al. have suggested a combined surgery for congenital tracheal stenosis and cardiac anomalies in infants after their experience of nine cases with no operative mortality. [8] Guerriero et al. have reported a series of 47 patients who underwent contemporary surgical treatment for a cardiac disease and an associated noncardiac lesion usually represented by cholelithiasis or hernial defects with a mortality of 4.25%. [9]

Kouwenberg et al. have documented a mortality rate of almost 20% in patients operated for IM. [10] Risk factors for mortality included younger age, major CHD and ischemic bowel at the time of surgery.

From our successful perioperative management of obstructed TAPVC with midgut volvulus, we would like to suggest that combined surgical correction for major CHD and life-threatening IM may decrease the postoperative mortality and morbidity.


1Hoffman JI, Kaplan S. The incidence of congenital heart disease. J Am Coll Cardiol 2002;39:1890-900.
2Calzolari E, Garani G, Cocchi G, Magnani C, Rivieri F, Neville A, et al. Congenital heart defect : 15 years of experience of the Emilia-Romagna Registry (Italy). Eur J Epidemiol 2003;18:773-80.
3Stewart DR, Colodny AL, Willard CD. Malrotation of the bowel in infants and children: A 15 year review. Surgery 1976;79:716-20.
4Forrester MB, Merz RD. Epidemiology of intestinal malrotation, Hawaii, 1986-99. Paediatr Perinat Epidemiol 2003;17:195-200 .
5Pradat P, Francannet C, Harris JA, Robert E. The epidemiology of cardiovascular defects, Part I : A study from three large registries of congenital malformation. Pediatr Cardiol 2003;24:195-221.
6Torres AM, Ziegler MM. Malrotation of the intestines. World J Surg 1993;17:326-31.
7Messineo A, MacMillan JH, Palder SB, Filler RM. Clinical factors affecting mortality in children with malrotation of the intestine. J Pediatr Surg 1992;10:1343-5.
8Loukanov T, Sebening C, Springer W, Ulmer H, Hagl S. Simultaneous management of Congenital tracheal stenosis and cardiac anomalies in infants. J Thorac Cardiovasc Surg 2005;130:1537-41.
9Guerriero G, Renzulli A, Mallardo N, Bellitti R, Piccolboni D, Mazzei V, et al. A plea for combined cardiac and noncardiac surgery. Ital J Surg Sci 1983;13:187-91.
10Kouwenberg M, Severijnen RS, Kapusta L. Congenital cardiovascular deaths in children with intestinal malrotation. Pediatr Surg Int 2008;24:257-63.