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    Abstract
    Introduction
    Case Report
    Discussion
    References

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CASE REPORT  
Year : 2011  |  Volume : 14  |  Issue : 1  |  Page : 51-54
Use of extracorporeal membrane oxygenator support to salvage an infant with anomalous left coronary artery from pulmonary artery


1 Department of Cardiothoracic and Vascular Anesthesia, All India Institute of Medical Sciences, New Delhi, India
2 Department of Cardiothoracic Surgery, All India Institute of Medical Sciences, New Delhi, India

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Date of Submission16-Jun-2010
Date of Acceptance03-Sep-2010
Date of Web Publication31-Dec-2010
 

   Abstract 

Anomalous left coronary artery from pulmonary artery (ALCAPA) is a congenital acyanotic heart disease where the left coronary artery (LCA) arises from the pulmonary artery. This results in the LCA receiving blood supply from the low-pressure right ventricle having minimal extractable oxygen. The oxygen delivery to the left ventricle (LV) is severely hampered causing severe hypoxic LV dysfunction early in life. Early surgery prior to serious, irreversible LV dysfunction is the key to survival. Children with ALCAPA usually present in their first few weeks of life, with severe LV dysfunction. After surgical correction of the defect, the myocardium may not recover early from the presurgery myocardial dysfunction. We describe a case where extracorporeal membrane oxygenator was utilized as a means of ventricular support during this critical postoperative period resulting in a favorable outcome.

Keywords: Anomalous left coronary artery from pulmonary artery, extracorporeal membrane oxygenator, left coronary artery

How to cite this article:
Malik V, Pandey A, Chauhan S, Airan B. Use of extracorporeal membrane oxygenator support to salvage an infant with anomalous left coronary artery from pulmonary artery. Ann Card Anaesth 2011;14:51-4

How to cite this URL:
Malik V, Pandey A, Chauhan S, Airan B. Use of extracorporeal membrane oxygenator support to salvage an infant with anomalous left coronary artery from pulmonary artery. Ann Card Anaesth [serial online] 2011 [cited 2019 Aug 21];14:51-4. Available from: http://www.annals.in/text.asp?2011/14/1/51/74401



   Introduction Top


Anomalous left coronary artery from pulmonary artery (ALCAPA) was first described in 1866 by Brooks. [1] The first clinical description in conjunction with autopsy findings was described by Bland and colleagues in 1933, so the anomaly is also called Bland−WhiteGarland syndrome. [2]

The usual clinical course is of severe left-sided heart failure and mitral valve insufficiency presenting during the first few months of life. However, in some cases, collateral blood supply from the right coronary artery is sufficient and symptoms may be subtle or even absent. Arrhythmias or sudden cardiac death in adult life may be the first clinical presentation in patients with ALCAPA.

We report a case of a 2-month-old infant with ALCAPA with where extracorporeal membrane oxygenator (ECMO) was used as a bridge to recovery of the LV.


   Case Report Top


A 2-month-old infant weighing 3 kg, diagnosed to have ALCAPA, severe mitral regurgitation and severe LV dysfunction, and ejection fraction of 0.15. The child was the youngest of three siblings, with no family history of cardiac illness was taken up for transplantation of the left coronary artery from the pulmonary artery to the aorta.

No premedication was administered; the infant was transferred to the OR in a warm, humid, oxygen rich milieu. Preoperative inotropic support of intravenous infusion of dobutamine 5 μg/kg/min was continued through the transfer. electrocardiogram and pulse oxymetry monitoring was initiated prior to induction of anesthesia.

Anesthesia was induced with ketamine 2 mg/kg and muscle relaxation was obtained with rocuronium

1 mg/kg. The trachea was intubated with a 4.5 mm nasal endotracheal tube and was fixed at 13 cm at the nares.

Anaesthesia was maintained with repeated intravenous fentanyl bolus of 1−2 μg/kg up to 12 μg/kg, repeated intravenous midazolam 0.3 mg/kg and isoflurane. Neuromuscular blockade was maintained with vecuronium 0.1 mg/kg repeated every 30 min.

With the left coronary artery (LCA) arising from the pulmonary artery (PA), the left coronary perfusion pressure depends on the difference in pressures between the PA and LV. Higher pulmonary vascular resistance has to be maintained to promote perfusion of blood through LCA. The patient was ventilated with air oxygen mixture to achieve this goal.

Adequate heparinization with unfractionated heparin 4 mg/kg was obtained prior to commencing CPB.

Myocardial preservation during surgical correction was obtained with high potassium blood-crystalloid (4:1) cardioplegia solution. The solution was delivered by a ''Y'' shaped delivery system via two cardioplegia canulae, one inserted into the aorta and the other into the PA to perfuse the right and left coronary systems, respectively. The aorta and the PA were cross-clamped during the delivery to prevent the solution from perfusing the proximal systemic and pulmonary circulation.

Surgical repair with fashioning of coronary button from the PA and later anastamosis of the same to the proximal aorta was done. Adequate hemostasis was obtained at the aortic and PA suture lines. No attempt was made to correct the severe mitral insufficiency by the surgeon.

Attempted weaning from CPB, even with high inotropic support in the form of adrenalin 0.2 μg/kg/min, noradrenalin 0.2 μg/kg/min, dopamine 20 μg/kg/min, dobutamine 20 μg/kg/min, milrinone 1 μg/kg/min was unsuccessful. During these attempts at weaning off CPB, visually the heart appeared distended, there also were 3−4 runs of ventricular tachycardia which appeared with loading the LV and subsided once the load was taken off the left ventricle. A supportive CPB of more than an hour was tried. Inotropic support in the form of dopamine 5 μg/kg/min, dobutamine 5 μg/kg/min, and milrinone 0.5 μg/kg/min had to be continued to maintain perfusion pressures during supportive CPB. There was no evidence of any rhythm abnormality or ongoing myocardial ischemia during this period of supportive CPB. It was thus ascertained that myocardial support would be required before the native ventricular function would recover, and hence, a temporary ventricular support as a bridge to recovery in the form of ECMO would be required. No further attempts at weaning from CPB were made in an attempt to reduce any serious insult to the left ventricle recovering from chronic ischemia.

An ECMO circuit using Medtronic ECMO oxygenator (Medtronic Inc, Minneapolis, MN, USA) was assembled. The outflow and inlet circuit on ECMO is similar to the CPB circuit. The main difference between ECMO and CPB is in the oxygenator which is a true silicon rubber membrane allowing gas exchange for prolonged period of time and the reservoir which is a collapsible bladder in case of an ECMO circuit. The circulation was changed over from the CPB to the ECMO circuit. The cardiac output during the changeover period of about 15−20 s was maintained by the native heart. The infant was transferred with ECMO support to the intensive care unit (with sternum open). Blood flow of 320 ml/min using a roller pump, gas flow of 200 ml/ min, FiO 2 of 70% and temperature of 37 ºC was maintained, while the child was on ECMO support. The activated clotting time was maintained between 180 and 250 s.

The ventilatory strategy during this period when the patient was on ECMO was directed at reduction of lung atelectasis and avoidance of oxygen toxicity to the lungs. The child was ventilated with room air in pressure regulated volume control mode with a tidal volume of 5−7 ml/kg and a PEEP of 4 cm H 2 O.

Intravenous antibiotic prophylaxis in the form of injection Piperacillin 50 mg/kg + Tazobactum, 6.25 mg/kg (fixed dose combination) thrice daily, injection Vancomycin 10 mg/kg thrice daily, and injection Amikacin 0.5 mg/kg thrice daily was administered. The antibiotics were in line with institutional protocol as advised by the hospital infection-control committee from time to time.

The patient was on ECMO for five days postoperatively. An unsuccessful trial of weaning from ECMO was undertaken on the third postoperative day. The following day inotropic support was optimized and adrenalin and noradrenalin at the rate of 0.05 μg/kg/min were added in addition to dobutamine 5 μg/kg/min. A weaning trial was commenced later in the day with gradual reduction of the ECMO blood flow. The ventricular function improved dramatically, and the infant was successfully weaned off from ECMO support early on the fifth postoperative day. The inotropic support was also reduced and withdrawn over the ensuing 48 h. The infant was subsequently extubated after 72 h.


   Discussion Top


Infants with ALCAPA do not present prenatally because of the favorable fetal physiology that includes (1) equivalent pressures in the main pulmonary artery and aorta secondary to a non-restrictive patent ductus arteriosus, and (2) relatively equivalent oxygen concentrations due to parallel circulations. This results in normal myocardial perfusion and, therefore, no stimulus for collateral vessel formation between the right and left coronary artery systems is present during the fetal life.

Shortly after birth, as the circulation changes from a parallel situation to one in series, pulmonary artery pressure and resistance decrease. In the presence of ALCAPA this results in the perfusion of left ventricular myocardium by relatively desaturated blood under low pressure from the PA, leading to myocardial ischemia.

Initially, myocardial ischemia is transient, occurring during periods of increased myocardial demands, such as when the infant is feeding and crying. Further increases in myocardial oxygen consumption lead to infarction of the anterolateral left ventricular free wall. This often causes mitral valve papillary muscle dysfunction and variable degrees of mitral insufficiency. [3]

Collateral circulation between the right and left coronary systems ensues. Left coronary artery flow reverses and blood enters the pulmonic trunk from the LV through the LCA into the PA due to the low pulmonary vascular resistance (coronary steal phenomena). As a result, left ventricular myocardium remains underperfused. Consequently, the combination of left ventricular dysfunction and significant mitral valve insufficiency leads to congestive heart failure (CHF) symptoms (e.g., tachypnea, poor feeding, irritability, and diaphoresis) in the young infant. Inadequate myocardial perfusion likely causes significant chest pain and these symptoms of myocardial ischemia may be misinterpreted as routine infantile colic.

These patients present for surgery in the early neonatal life, thereby posing the dual challenges of the anesthetic management of a very small and sick infant and an ischemic LV myocardium for surgery. Early surgery and adequate recovery of the LV function is the key to survival.

ECMO is a term used to describe prolonged (days to weeks) mechanical support for patients with reversible heart or lung failure. The technology is similar to cardiopulmonary bypass as used during cardiac surgery, only modified for prolonged use at the bedside intensive care unit. ECMO is capable of effectively and safely supporting respiration and circulation in neonates with severe reversible respiratory failure and a moribund clinical presentation. Such neonates usually have very minimal chances of survival which on ECMO support are increased considerably and may approach more than 60%. [4] This system provides excellent oxygen saturation and carbon dioxide removal. When applied early in the course of severe failure, newborns who would have otherwise died will regularly survive. The indications for use of ECMO in neonates include all those conditions where there is a respiratory/ cardiac compromise which may recover with time, [4] e.g., meconium aspiration syndrome, congenital diaphragmatic hernia, following ALCAPA repair, following arterial switch operation in infants with borderline ventricle, failure to wean from CPB following definitive surgery.

The cardiac function usually recovers over a period of time as the left coronary artery is now connected to the aorta. [5] The use of ECMO acts as a biventricular assist device and supports the infant during this crucial time required for recovery of ventricular function. [6]

The infant in this case was on ECMO support for 120 h following which his native ventricular function had recovered adequately to be weaned off from support.

 
   References Top

1.Brooks HS. Two cases of an abnormal coronary artery of the heart arising from the pulmonary artery. J Anat Physiol 1885;20:26-9.  Back to cited text no. 1
    
2.Bland EF. Congenital anomalies of the coronary arteries: Report of an unusual case associated with cardiac hypertrophy. American Heart Journal 1933,8:778-80.  Back to cited text no. 2
    
3.Rein AJ, Colan SD, Parness IA, Sanders SP. Regional and global left ventricular function in infants with anomalous origin of the left coronary artery from the pulmonary trunk: Preoperative and postoperative assessment. Circulation 1987;75:115-23.  Back to cited text no. 3
    
4.Lequier L. Extracorporeal life support in pediatric and neonatal critical care: A review. J Intensive Care Med 2004;19:243-58.  Back to cited text no. 4
    
5.Shivalkar B, Borges M, Daenen W, Gewillig M, Flameng W. ALCAPA syndrome: An example of chronic myocardial hypoperfusion. J Am Coll Cardiol 1994;23:772-8.   Back to cited text no. 5
    
6.Chaturvedi RR, Macrae D, Brown KL, Schindler M, Smith EC, Davis KB, et al. Cardiac ECMO for biventricular hearts after paediatric open heart surgery. Heart 2004;90:545-51.  Back to cited text no. 6
    

Top
Correspondence Address:
Anil Pandey
Cardiothoracic Sciences Centre, All India Institute of Medical Sciences, Ansari Nagar, New Delhi 110 029
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0971-9784.74401

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