Anaesthetic Management of A Rare Case of Hemianomalous

Pulmonary Venous Connection

Case Report

A 11 month old malnourished baby boy, weighing 5kgcame with complaints of failure to thrive and recurrentURI. His general physical examination was normal andcardiovascular examination revealed a systolic murmurand heart sounds were of equal intensity on both sides ofthe chest. His routine investigations were normal withhemoglobin of 14 g%. His chest X-ray showeddextrocardia, cardiomegaly and pulmonary plethora. HisECG showed upright r-waves in V1. 2D Echo showed situssolitus, mesocardia, bilateral superior venacava, dilatedcoronary sinus, cardiac TAPVC, perimembranous VSD,ostium secundum ASD (R→L), severe PAH, dilatedpulmonary artery, severe TR.

Patient was posted for total intracardiac repair undergeneral anaesthesia and cardiopulmonary bypass.The babywas given intramuscular ketamine 25 mg and atropine0.05mg and shifted to OT. Routine monitors wereconnected. His saturation on room air was 86%. In the OTgeneral endotracheal anaesthesia was given with no 4(UNCUFFED) tube after inducing with inj. fentanyl20mcg, inj. Midazolam 1mg and inj.vecuronium 1mg iv.During preoxygenation the saturation increased to 94%and with intubation attained 100%. Left femoral arterialline was secured with 20G iv cannula and left radialarterial line was secured with 24 G iv cannula. Rightinternal jugular vein was secured with 5 french, 8 cmcatheter. Maintenance was with 100% oxygen, isoflurane0.8-1% and vecuronium.

After heparinisation (2500U) and obtaining asatisfactory ACT patient went on CPB. Peroperativefindings were as follows-absent innominate vein, bilateral

ANAESTHETIC MANAGEMENT OF A RARE CASE OFHEMIANOMALOUS PULMONARY VENOUS CONNECTION

KR Manjunath*, S Shwetha Odeyar**, Murali Krishna***, Shyam P Shetty***and Keshav Murthy****

*Consultant Cardiac Anaesthetist, **PG Student in Anaesthesia, ***Consultants in Cardio thoracic Surgery,****Consultant Cardiologist, Apollo BGS Hospitals, Mysore 570 023, India.

Cooespondance to: Dr KR Manjunath, Consultant Cardiac Anaesthetist, Apollo BGS Hospitals, Mysore 570 023, India.

We present a case report on anaesthetic management of a case of hemianomalous pulmonary venousconnection with VSD, ASD for total intracardiac repair. A balanced anaesthetic technique was used withoxygen, isoflurane, fentanyl, midazolam,vecuronium.Patient was successfully operated undercardiopulmonary bypass with hypothermia.

Key words: Hemianomalous pulmonary venous connection(HAPVC), Atrial septal defect(ASD).

SVC, left atrial isomerism, situs solitus withdextroversion, ostium secundum ASD, dilated coronarysinus, left pulmonary veins draining into left atrium, rightpulmonary veins draining into right atrium near thecoronary sinus, perimembranous VSD, large pulmonaryartery and normal relation of great arteries. Had the rightpulmonary veins drained into coronary sinus directly, itwould have been a real challenge to re-route thepulmonary veins into left atrium as left superior vena cavawas draining into coronary sinus. As there was left atrialisomerism we expected that sinoatrial node was located inan unusual location with high possibility of developingheart blocks. On full CPB, aorta cross clamped and coldblood cardioplegia given. Patient was cooled to 28ºC,pediatric membrane oxygenator used (minimax plus),nonpulsatile flow achieved and hemofiltration done and550 mL of water removed. ASD enlarged and rightpulmonary veins were routed through the enlarged ASDinto the left atrium.VSD was closed with a Dacron patchand right atrium was closed. Heart was deaired and crossclamp released. Heart started beating in sinus rhythm (120bpm), came off bypass with milrinone and noradrenaline.Before heparin reversal, RA/PA oxygen step up waschecked with ABG of RA & PA blood samples. There wasno significant step up of oxygen saturation from RA toPA. Post bypass pressure in pulmonary artery was onethird of systemic pressure. Heparin was reversed withprotamine & surgery completed.

Patient was shifted to ICU, post operative analgesiawas maintained with fentanyl (10 mcg/hr) infusion. Afterconfirming that ABG, serum electrolytes includingcalcium, chest X-ray, blood glucose were normal with no

147 Apollo Medicine, Vol. 8, No. 2, June 2011

Apollo Medicine, Vol. 8, No. 2, June 2011 148

Case Report

drainage from ICD’s - patient was fully rewarmed,reversal given with neostigmine and atropine and patientwas weaned and extubation done 6 hours after surgery.

DISCUSSION

This is a very rare case of cyanotic congenital heartdisease with VSD, HAPVC of right pulmonary veinsdraining near the coronary sinus into the right atrium,ASD (R→L), PAH, TR, left atrial isomerism with leftsuperior vena cava draining into coronary sinus [1]. If alung infection develops in normal lung (e.g. left lung inright HAPVC) then a picture similar to TAPVC withsevere desaturation and hypoxia would develop.Anaesthetist plays an optimal role in managing such cases

wherein the pulmonary mechanics and respiratory care areof utmost importance [2-3].

REFERENCES

1. Raisher BD,Grant JW, Martin TC, et al. Complete repairof TAPVC in infancy, Journal of thoracic cardiovascularsurgery 1992;104: 443-448.

2. Sandeep Khanna, Minati choudhury, Usha Kiran.TAPVC: post operative problems and management.Indian Journal of Anaesthesia 2009; 53(1): 71-74.

3. David L Tomlinson, Hiroshi Goto, James C Graf,Korasami Arakawa. Incidental discovery of persistent leftsuperior vena cava during cardiac surgery. AnesthesiaAnalgesia, 1989; 69: 393-395.

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