Learning the abc of ALCAPA
September 19, 2019 | Contributed by R Srivatsan
Are you familiar with what ALCAPA is? Have you ever heard of this particular type of congenital heart disorder?
As a Foundation working for children who suffer from such ailments, it is important to understand every detail of the cases that come to us, especially complex ones of ALCAPA.
Symptoms of ALCAPA
- Crying or sweating during feeding
- Pale skin
- Poor feeding
- Rapid breathing
What is ALCAPA?
It is a condition where the left main coronary artery rises from the pulmonary artery instead of the aorta. It supplies oxygen rich blood to the left side of the heart and the mitral valve. Here, collaterals arising from the right coronary artery feed the left coronary artery territory.
Babies born with ALCAPA can go undetected as the pulmonary artery resistance is high. So, as the child grows this resistance falls and leads to reverse flow from the left coronary artery back to the pulmonary artery. This causes muscle failure of the heart which can later lead to cardiac dysfunction.
This condition was first described by Bland, White and Garland (1933) and hence it is also commonly knownas Bland White Garland syndrome. Out of all CHDs the occurrence of ALCAPA is 0.2-0.4% making it a rare congenital heart defect.
A good pediatrician can often suspect this rare condition through detection of a murmur and ECG changes. The only treatment for this condition is surgical correction.There are various techniques of operation but the best result is obtained by connecting back the left coronary artery from the pulmonary artery to the aorta. The defect in the pulmonary artery is closed with a tanned pericardial patch. In those patients with severe ventricular dysfunction the sternum is often kept open and closed after 24 hours following surgery.
As rare as this disease is, the corrective surgery is considered a high-risk surgery with mortality in different series showing around 10 to 20%. It is high-risk as these children have severe left ventricular failure pre-operation. It takes around one year after the operation for the ventricular function to recover.
Many studies have shown that many of these children post-surgery will require a period of ECMO support. This is one condition where children following cardiac surgery take a very long time in ICU for recovery. The post-operative mortality is usually due to acute cardiac failure.
Long term follow-up shows that in most of these patients the left ventricular function returns to near normal within a year time and then they lead a near normal life.
Genesis Foundation saving lives of ALCAPA diagnosed children
Over the years we have supported various types of complex CHD, including ALCAPA cases. To date, seven children with this particular CHD have had a chance at life again as we have been able to finance their intervention.
Baby of Mitra and C Praneeth are two such cases.
Baby of Mitra was only five months old when she was detected with ALCAPA. She was born in Thrissur, Kerala to Mahesh and Mitra. Mahesh looks after the family through his work which can be a little inconsistent as he is a daily wage worker. He earns Rs 4000 per month whenever work is available. She had severe left ventricular dysfunction and was shown at a private hospital in Thrissur itself where this particular CHD was detected. She required urgent repair to live and for that she needed to be transported in an ambulance to Amrita Institute of Medical Sciences (AIMS) in Kochi. Mahesh confided in the doctors about the medical costs being too high for the family to bear. AIMS, our partner hospital reached out to us for support and through our CSR collaboration with Pernod Ricard India Foundation we were able to save her life and ensure that she got the appropriate and timely intervention for ALCAPA.
C Praneeth, is the only child born to Nevema and Chellapa from Tiruchirappalli, in Tamil Nadu. All was okay until the boy was around two months old. He became extremely ill with a severe bout of pneumonia. He was hospitalized and this is when the doctors observed that he had dilated cardiomyopathy, which is enlargement of the left ventricle. He was discharged and put on medication and follow-up. Time passed and when they returned for a follow-up, the medication was changed. In their hearts, both parents knew the problem could not be taken lightly so then went for a second opinion. They were referred to Miot Hospital in Chennai where ALCAPA was detected. He required open-heart surgery but Chellapa could not afford the high cost of surgery. He is a photographer earning Rs 5000 per month.
State health insurance and a private donor provided them relief, but they still required financial aid to meet the full costs. We were able to support this case and give the boy a chance to beat the rare disease.
As a Foundation working for children, it in our endevaour to save as many children with critically ill congenital heart defects. We are always on the look-out to support families where little ones not only suffer the brunt of CHD but are diagnosed with a rare and complex type which needs immediate care. We pride ourselves in working with the best medical practices in the country who share the same vision and commitment as us to truly make a difference in this country, one heart at a time.