Parturient with double outlet right ventricle without pulmonary stenosis: Perioperative management

Akhil Kant Singh; PM Singh; Shubhangi Arora; Vimi Rewari; Anjan Trikha

doi:10.4103/2249-4472.104738

CASE REPORT

Year : 2012 | Volume : 2 | Issue : 2 | Page : 109-111

Parturient with double outlet right ventricle without pulmonary stenosis: Perioperative management

Akhil Kant Singh, PM Singh, Shubhangi Arora, Vimi Rewari, Anjan Trikha
Department of Anaesthesia, All India Institute of Medical Sciences, Delhi, India

Date of Web Publication

17-Dec-2012

Correspondence Address:
P M Singh
Department of Anaesthesia, All India Institute of Medical Sciences, Delhi
India

Source of Support: None, Conflict of Interest: None

Check

DOI: 10.4103/2249-4472.104738

Abstract

Double outlet right ventricle (DORV) is a rare cardiac condition in which the heart demonstrates single ventricle physiology. The management of these patients depends upon the presence or absence of pulmonary stenosis. They can present as congestive cardia failure on one end and as cyanotic disease on the other extreme. We present a case of DORV without pulmonary stenosis for cesarean section and highlight specific management issues in these patients.

Keywords: Double outlet right ventricle, double outlet right ventricle for cesarean section, single ventricle physiology

How to cite this article:
Singh AK, Singh P M, Arora S, Rewari V, Trikha A. Parturient with double outlet right ventricle without pulmonary stenosis: Perioperative management. J Obstet Anaesth Crit Care 2012;2:109-11

How to cite this URL:
Singh AK, Singh P M, Arora S, Rewari V, Trikha A. Parturient with double outlet right ventricle without pulmonary stenosis: Perioperative management. J Obstet Anaesth Crit Care [serial online] 2012 [cited 2022 Aug 16];2:109-11. Available from: https://www.joacc.com/text.asp?2012/2/2/109/104738

Introduction

Double outlet right ventricle (DORV) is a rare cardiac condition in which the heart demonstrates single ventricle physiology. DORV refers to any cardiac anatomy in which both the aorta and pulmonary trunk originate from the right ventricle. Both the systemic and pulmonary circulations are in parallel, and thus a delicate balance exists between the two. Any alteration in one circuit leads to opposite change in blood flow in the other circulation. A change in pulmonary circulation can bring about cyanosis on one end to frank congestive failure on the other end. ^[1] Anesthesia for these patients needs a through understanding of the physiology of DORV in order to maintain stable hemodynamics. We present successful anesthetic management of a pregnant parturient for cesarean section and also highlight the physiological aspects in the management.

Case Report

A 25-year-old female, known case of DORV at 37 weeks of gestation in labor was scheduled for elective cesarean section in view of associated severe pulmonary artery hypertension (PAH). She was a booked case, currently NYHA II with right ventricular hypertrophy evident on electrocardiograph [Figure 1], and echocardiography showed situs solitus, DORV, large subaortic ventricular septal defect (VSD), severe PAH, dilated left atrium, and normal ventricular function. Hemogram showed hemoglobin of 9.5 gm% ; rest of the investigations and coagulation profile were within normal limits. Physical examination revealed pulse rate of 91 per minute, BP of 114/74 mm Hg, and room air oxygen saturation of 96%.

Figure 1: Preoperative electrocardiograph showing right ventricular hypertrophy

Click here to view

A preoperative room air arterial blood gas sampling showed a pO ₂ of 74 mm Hg and pCO2 of 39 mm Hg.

She denied any previous history of cyanosis; however, she had multiple hospital admissions due to recurrent chest infections for which she was on occasional diuretics. No past history suggestive of palpitations or any syncope could be elicited. Her functional status and symptoms remained as pre-pregnancy and did not show any worsening with pregnancy.

After obtaining a written informed consent and explaining the possibility of postoperative intensive care need, the patient was shifted to operating room where all devices for routine monitoring were connected. Room air oxygen saturation was noted to be 96%. Left radial artery cannula was secured for invasive arterial blood pressure monitoring. Co-loading with around 300 ml of Ringer's lactate was done simultaneously. A combined spinal epidural technique was planned using needle through needle technique. Intrathecally, 7.5 mg bupivacaine 0.5% heavy was administered along with 25 μg of fentanyl, making a total volume of 2 ml, at the L3-L4 interspace in sitting position. Sensory level till T8 was detected on making the patient supine; the level was extended till T6 by lowering head end. After ensuring adequate level of block, the surgery was allowed to proceed. Milrinone and phenylephrine were kept at hand during the procedure. The patient was hemodynamically stable throughout the procedure. The minimum blood pressure noted was 92/68, which responded to another fluid bolus of 200 ml Ringer's lactate. At the end of the procedure, 10 ml of 0.125% bupivacaine and 3 mg morphine were given through the epidural catheter for postoperative analgesia. The patient was shifted to the PACU for monitoring.

Discussion

Although the frequency is not well documented, the incidence of DORV is probably less than 1 in 3000. ^[2] Physiologically, DORV is classified into subtypes on the basis of associated VSD. ^[3],[4] The diagnosis frequently overlaps with other conditions, which are classified solely as DORV. A major deciding factor for a DORV to be cyanotic versus non-cyanotic depends upon the presence of associated pulmonary stenosis which limits the blood flow to the lung. Consequently, this is the pivot on which the anesthetic management rests.

Our patient had DORV without pulmonary stenosis. She also had a history of frequent pulmonary infections. The primary issue in such a patient would be increased pulmonary blood flow, and the patients often present with pulmonary congestive features. Keeping this in mind, we chose to cautiously co-load the patient with 300 ml of lactated Ringer. In such patients, a small fall in pulmonary flow would prove to be beneficial as it lowers the circulatory load of overperfused pulmonary circulation. We chose to administer a combined spinal epidural as it allows the use of smaller doses of spinal local anesthetics which reduce the incidence of sudden and severe drop in the blood pressure. In addition, the sympathectomy achieved would help in the reduction of pulmonary congestion.

Milrinone and phenylephrine were kept ready as rescue drugs to treat intraoperative cyanosis due to significant fall in pulmonary blood flow as a result of shunting of blood to systemic circulation (parallel circulation of single ventricle physiology). Milrinone, being an inodilator, would increase the cardiac output and cause dilation of the pulmonary vasculature. ^[5] Phenylephrine would have caused systemic vasoconstriction and diverts the blood flow to the pulmonary circulation.

On the contrary, in patients with associated pulmonary stenosis, general anesthesia would be the preferred technique as it prevents the fall in systemic vascular resistance due to sympathectomy secondary to neuraxial block. Patients with DORV have systemic and pulmonary circuits in parallel. ^[6] The circuit with lower resistance would preferentially be perfused. ^[7] A fall in systemic vascular resistance (SVR) in these patients leads to further reduction in pulmonary blood flow, which impedes oxygenation.

Therefore, it is imperative to tailor the anesthetic management according to the specific needs of the patient, which will depend on the particular morphology of the cardiac lesions. DORV is a constellation of pathologies ^[8] and a safe anesthetic technique must take into consideration this specific combination of lesions.

References

1.	Cosgrove MS. Anesthesia for the adult patient with an unrepaired congenital cyanotic heart defect: A case report. AANA J 2012;80:197-203. [PUBMED]
2.	Saleeb SF, Juraszek A, Geva T. Anatomic, imaging, and clinical characteristics of double-inlet, double-outlet right ventricle. Am J Cardiol 2010;105:542-9. [PUBMED]
3.	Cevik A. Double outlet right ventricle: Fallot type or non-Fallot type. Anadolu Kardiyol Derg 2012;16???.
4.	Bartelings MM, Gittenberger-de Groot AC. Morphogenetic considerations on congenital malformations of the outflow tract. Part 2: Complete transposition of the great arteries and double outlet right ventricle. Int J Cardiol 1991;33:5-26. [PUBMED]
5.	Krushansky E, Burbano N, Morell V, Moguillansky D, Kim Y, Orr R, et al. Preoperative management in patients with single-ventricle physiology. Congenit Heart Dis 2012;7:96-102. [PUBMED]
6.	Krishna R, Umesh G. Anesthetic management of caesarean section in a patient with double outlet right ventricle. J Obstet Anaesth Crit Care 2012;1:50-2.
7.	Yuki K, Casta A, Uezono S. Anesthetic management of noncardiac surgery for patients with single ventricle physiology. J Anesth 2011;25:247-56. [PUBMED]
8.	Lagopoulos ME, Manlhiot C, McCrindle BW, Jaeggi ET, Friedberg MK, Nield LE. Impact of prenatal diagnosis and anatomical subtype on outcome in double outlet right ventricle. Am Heart J 2010;160:692-700. [PUBMED]