Anesthetic Considerations in an Adult Congenital Rubella Syndrome-Patent Ductus Arteriosus Ligation in a Resource-Limited Setting

Dear Editor,

Patent Ductus Arteriosus (PDA) should be corrected at an early age; however, it is a rare finding in some patients who survive into adult life without correction. Around 32% of Congenital Rubella Syndrome (CRS) patients have congenital heart defects (CHDs); the most prevalent CHD being PDA.[1]

A 31-year-old female with known CRS-PDA was posted for surgical PDA ligation because of the absence of transcatheter closure facility at our center. Electrocardiography revealed sinus tachycardia and left ventricle volume overload pattern [Figure 1]. Chest X-ray showed cardiomegaly with pulmonary plethora. The transthoracic echocardiography revealed 6 mm unrestrictive PDA (left to right shunt) with a gradient of 120 mm Hg and moderate pulmonary hypertension.

Figure 1.

Electrocardiography showing sinus tachycardia and left ventricle volume overload pattern (deep q waves and increase in R wave amplitude in leads V5 and V6)

Anesthetic management becomes challenging in these adult CRS-PDA patients due to alterations in cardiopulmonary physiology and multisystem abnormalities. In our case, a detailed preoperative anesthetic check-up was done as PDA patients are prone to arrhythmias, tachycardia, hypertension, myocardial ischemia, heart failure, bacterial endocarditis, alteration in pulmonary vascular status, lower respiratory tract infection, and poor weight gain. Apart from CHD concerns, CRS patients should be evaluated for sensorineural deafness, eye defects, unanticipated airway abnormalities, thrombocytopenia, hypothermia, and hypoglycemia.[2] Our patient had developmental delay and sensorineural deafness. Communication with the patient was a problem and therefore parental consent was taken. The operating room was kept ready with a defibrillator, emergency cardiac drugs, and difficult airway cart. American Society of Anesthesiologists (ASA) standard monitors were attached with an additional SpO₂ probe (one for the lower limb and another for the right upper limb). The importance of pulse oximeter at lower extremity during test clamping just before PDA ligation is to prevent inadvertent ligation of aorta, left pulmonary artery, or left main bronchus. A decrease in saturation should be communicated immediately to the surgeon during test clamping.

The patient was induced with intravenous fentanyl, midazolam, etomidate, and vecuronium. Utmost care was taken to avoid factors worsening pre-existing pulmonary hypertension. Anesthesia was maintained with oxygen: air, sevoflurane, fentanyl, and vecuronium. We kept low FiO₂ and PaCO₂ between 40 and 50 mmHg to reduce left to right shunting. Left femoral artery was cannulated and right internal jugular venous catheter was also introduced. Body warmer was used to prevent hypothermia and blood glucose levels were also checked to prevent hypoglycemia. Deliberate hypotension is mandatory to lower the tension on vessels to subside the risk of PDA tear and to prevent injury to the phrenic or recurrent laryngeal nerve. Therefore, systolic blood pressure was maintained in the range of 60-80 mmHg with titrated Nitroglycerine (NTG) infusion (0.5 ugs/kg/min - 2.0 ugs/kg/min) during PDA dissection and ligation. Rise in diastolic blood pressure confirmed successful PDA ligation. Intercostal block was administered for postoperative analgesia. The patient was positioned supine and transthoracic echocardiography revealed no residual leak. After successful extubation, the patient was shifted to the intensive care unit.

Literature review reveals that during the simultaneous presence of CRS and PDA, there is difficulty in satisfactorily placing and stabilizing a prosthesis and a risk of embolization during release; therefore, surgical ligation should be preferred over transcatheter closure in CRS-PDA patients.[3] A scoping review of the literature identified only two case reports describing anesthetic management of adult PDA patients. Wani Z et al. describes anesthetic management of large PDA closure and coarctation of the aorta under total hypothermic circulatory arrest.[4] Another case report describes anesthetic management during surgical interruption of an adult calcified PDA.[5] To the best of our knowledge, this is the first report describing anesthetic management of an adult PDA ligation in a patient with CRS.

Sound knowledge of pathophysiological changes associated with the CRS and PDA, adapting anesthetic principles of PDA ligation from pediatric to adult and optimum utilization of available resources helped in the successful management of a long-neglected failure to thrive condition.

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Conflicts of interest

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