Anaesthetic Management of Uncorrected DORV with Cerebellar Abscess: A Balancing Act

Dr. Adethen Gunasekaran MD, DNB, DM*; Dr. Jeevasri Calaidrajane MD; Dr. Vivekchandar Chinnarasan MD.DM; Dr. Jerry Jame Joy MD, DM

Senior Resident, Department of Anaesthesia & Critical Care, JIPMER Puducherry.

*Corresponding author

Dr.Adethen Gunasekaran, Senior Resident, Department of Anaesthesia & Critical Care, JIPMER Puducherry.

Abstract

The incidence of brain abscesses in cyanotic congenital heart disease patients is high due to defects in embryonic development. The associated congenital anomalies include Tetralogy of Fallot (TOF), DORV, and transposition of the great vessels. The complex anatomy and physiology of these conditions is critical for providing anesthesia to such patients. Key considerations during the perioperative workup involves the through understanding of the complex anatomy, the presence of associated anomalies and its physiological changes. The pathophysiology of DORV depends on multiple factors. Here, we discuss a case of 9-year-old child with uncorrected DORV who developed cerebellar abscess. The anaesthetic challenges encountered and the strategies employed during the case have been detailed.

Introduction

The modern era has seen remarkable advancements in diagnosing and managing congenital heart diseases (CHDs). However, complex CHDs like double outlet right ventricle (DORV) continue to present unique challenges for anaesthesiologists. Here, we discuss the case of a 9-year-old child with uncorrected DORV who developed a cerebellar abscess. The anaesthetic challenges encountered and the strategies for successful management.

Case Report

A 9-year-old child presented with a month-long history of headache, vomiting, and fever. The child was diagnosed with congenital heart disease at birth due to cyanotic spells and recurrent lower respiratory tract infections (LRTIs). Further evaluation revealed a Double Outlet Right Ventricle (DORV) with Ventricular Septal Defect (VSD), pulmonary atresia, and Major Aortopulmonary Collateral Arteries (MAPCA) dependency. There were no other associated congenital anomalies. On examination, the child weighed 21 kg, with a Glasgow Coma Scale (GCS) score of 15 and stable vital signs: heart rate of 117 beats per minute, blood pressure of 90/60 mmHg, and oxygen saturation (SpO2) of 75% in room air. Clubbing was present, but there were no gross facial anomalies. Haemoglobin levels were 13.4 g/dL, and other investigations were within normal limits. An echocardiogram revealed that the pulmonary artery and the aorta were arising from the right ventricle, with a large subaortic VSD. .NCCT and MRI revealed multiple ICSOL in the cerebellum with enhancement`of the margins.( Fig 2A and 2B)

Figure 1: DORV-TOF type

Figure 2:
2A: NCCT Brain

2B: MRI Brain showing R cerebellar abscess

The child was diagnosed with a cerebellar abscess and underwent posterior cranial fossa decompression with abscess evacuation as the first surgical intervention. After standard monitors (SpO2, non-invasive blood pressure, ECG, and temperature) were attached, baseline parameters were noted. Anaesthesia was induced with ketamine(2mg/kg), vecuronium(0.1mg/kg), and sevoflurane(upto 3%). A 5.5-sized micro cuffed PVC endotracheal tube (ETT) was secured. After confirming its position, the patient was put on mechanical ventilation in volume-controlled mode with a fraction of inspired oxygen (FiO2) of 40%, tidal volume of 180 ml, respiratory rate of 14 breaths per minute, positive end-expiratory pressure (PEEP) of 5 mmHg, and a peak airway pressure of 16 mmHg. An arterial line was secured in the right radial artery, and a central line was placed in the right internal jugular vein. Anaesthesia was maintained with sevoflurane and vecuronium, and fentanyl boluses were administered for analgesia. Saturation was maintained between 82-89% throughout the procedure. Intraoperatively, ceftriaxone 1g, tranexamic acid 300 mg, and paracetamol 300 mg were administered. The cerebellar abscess was successfully explored and excised, but the patient was not extubated on the operating table due to cerebellar oedema. Extubation was performed on postoperative day 1, and the patient was discharged a week later after completing a course of antibiotics.

Discussion

DORV has an incidence of 1 in 10,000 live births. The incidence of brain abscesses in cyanotic congenital heart disease patients ranges from 5% to 18.7%, often due to defects in the conotruncal stage of embryonic development. The associated congenital anomalies include Tetralogy of Fallot (TOF), DORV, and transposition of the great vessels1-3. Understanding the complex anatomy and physiology of these conditions is critical for managing such cases. Key considerations during the perioperative workup include the location of the VSD in relation to the great arteries, the relationship between the great arteries themselves, the morphology of the ventricles and their outflow tracts, and the presence of associated anomalies.

The pathophysiology of DORV depends on the VSD's location, aortic or pulmonary stenosis, and the outflow of the great arteries. Since systemic and pulmonary circulations are in parallel, the circuit with lower resistance is preferentially perfused1. Our case represents a TOF-type DORV (Fig 1), with clinical findings consistent with that subgroup. The presence of a systolic murmur, a history of cyanotic spells, and decreased saturation levels (SpO2 82%) confirmed the diagnosis. Brain abscesses are common in these patients due to factors such as bypassing pulmonary circulation, which decreases filtration by alveolar phagocytes. Secondary issues like polycythaemia, hypoxemia, and acidosis can lead to under perfusion in specific brain areas, promoting the growth of microorganisms4.

The anaesthetic goals in managing these cases include maintaining cardiac output, sustaining or increasing systemic vascular resistance (SVR), and preventing a fall in pulmonary vascular resistance (PVR). PVR should be kept low to maintain blood flow to the lungs. Other goals include maintaining cerebral perfusion pressure (CPP) and ensuring adequate depth of anaesthesia. Ketamine was used for anaesthetic induction to maintain cardiac output and increase SVR, while nitrous oxide was avoided due to uncertainty about pulmonary hypertension. Phenylephrine boluses were used to maintain blood pressure and SVR. PVR was managed by increasing oxygen concentration, hyperventilating to induce metabolic alkalosis, and avoiding hypothermia, hypoxemia, and acidosis. Culture-specific antibiotics were administered for a sufficient duration to ensure complete perioperative recovery4,5.

Conclusions

The successful anaesthetic management of this patient with uncorrected DORV and cerebellar abscess underscores the importance of a thorough understanding of the patient's cardiac physiology and the potential complications associated with their condition. A multidisciplinary approach, including careful hemodynamic monitoring and judicious use of anaesthetic agents, is essential for optimizing patient outcomes in such complex cases.

Declaration of patient consent: The authors certify that they have obtained all appropriate consent forms from the parents of the patient. In the form, the parent/s has/has given consent for their child’s images and other clinical information to be reported in the journal. The parent/s understands that her name and initial will not be published and due efforts will be made to conceal her identity, but anonymity cannot be guaranteed.

Financial support and sponsorship: Nil.

Conflicts of interest: There are no conflicts of interest.

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