What is CHD?
CHD refers to congenital heart disease in children. CHD refers to any heart abnormality present at birth or before birth [Fetal CHD]. CHD occurs in utero when the baby's heart is forming. Heart abnormalities can range from minor valve abnormalities to major structural malformations. Most CHD are diagnosed in infancy or childhood. Rarely certain CHD like ASD, Bicuspid valve, MVP can go undetected and present in adolescence or adulthood.
Other group of heart diseases affecting children—Acquired heart diseases which include
- Rheumatic heart diseases
- Kawasaki disease
- Myocarditis/ DCM d] Cardiomyopathies
- Other system diseases secondarily affect heart
Can we prevent CHD?
In most cases we don’t know the cause of CHD. Control of diabetes, avoid smoking, alcohol and other teratogenic drugs in pregnancy in in first trimester can reduce CHD in specific cases. Rubella vaccination and folate supplementation in prospective mothers can reduce CHD incidence in long –term.
What is critical CHD?
Many CHD in clinical practice are simple, easy to diagnose and can be treated easily, many of the simple CHD resolve on their own with time and growth of the child. 25 % of CHD are critical CHD. Critical CHD needs trans catheter intervention or surgery within the first year of life otherwise these babies will die. Most serious or critical CHD present early in life and are diagnosed before birth or in the early neonatal period. Critical CHD needs comprehensive diagnostic evaluation by pediatric cardiac team and will require open heart surgery in most cases before child’s first birthday.
What is GUCH/ Adult CHD?
GUCH-refers to grown up with CHD. In our country we see first time diagnosis of CHD in adulthood very commonly. GUCH is an important subspecialty usually managed by pediatric cardiologist or adult cardiologists with special training. In many countries GUCH population has overtaken CHD in children. GUCH population constitutes
- Uncorrected adult CHD
- Palliated CHD
- Repaired CHD in childhood
- Pregnancy with CHD
How to diagnose CHD?
Clinical presentation: Most commonly CHD is diagnosed after birth. Commonly they present in NICU with
- Cyanosis [low Spo2]
- Failed pulse oximetry screen
- Respiratory distress-mimicking lung disease
- Shock
- Isolated murmur
Older children: a] Heart failure b] Failure to thrive c] Cyanosis d] Exercise intolerance e] Cardiac murmur f] Palpitations/ Abnormal rhythm f] Syncope g] Recurrent lower respiratory tract infections
How to diagnose CHD?
- Investigations: In most cases of CHD diagnosis is confirmed by Echocardiography [Cardiac ultrasound]. In Smaller children, infants and neonates 2D echo will provide all the anatomical and physiological information regarding heart defect based on which Catheter or surgical interventions are planned.
- Cardiac CT scans: Usually required for complex lesions and planning redo surgical cases. Also when more information regarding extra cardiac anatomy like-Hilar PA, pulmonary veins, coronary anatomy, PA confluence, MAPCA we need higher investigations like cardiac CT/ CMRI.
- Diagnostic catheterization: Earlier was done in every case prior surgery. Now days mostly used in borderline cases for operability and demonstrate PAH and closure of abnormal vessels prior surgery.
How to manage CHD?
Medical / Trans catheter interventional treatments:
Children with CHD need lifelong care and need to be managed by specially trained doctors -pediatric cardiologist. Once CHD diagnosis is confirmed a comprehensive evaluation is done and short term and long management strategy is charted. Pediatric cardiac team will discuss each case with the PED CTVS team and the best treatment modality for the child based on anatomy and hemodynamics will be provided. CHD treatment can be medical, surgical, trans catheter interventional therapy.
- Medical treatment: Heart failure, cardiomyopathies, pulmonary hypertension, Kawasaki disease, arrhythmias
- Trans catheter interventional treatments: Closure of L->R shunts, ASD, VSD, PDA, A-P window
- Opening of stenotic lesions: Pulmonic stenosis, Aortic stenosis, COA, Mitral stenosis, branch PA stenting
- Closure of abnormal vessels: MAPCA, APC, coronary fistulas
How to manage CHD?
Cardiac surgery - In about 60% CHD cases-- cardiac surgery is the only option available. Cardiac surgery is either corrective or palliative in some cases. In about 10% cases child may require multiple surgeries in a staged manner due to anatomic constraints. Most important complications of operating in very small babies are bleeding, post-operative sepsis and heart block. In children open heart surgery by sternotomy is preferred due to smaller size. Adolescents older children few cardiac lesions can be corrected by minimally invasive cardiac surgery[MICS]. Advances in cardiac surgery and better understanding cardio pulmonary bypass [CPB] has resulted in very good outcomes after pediatric cardiac surgery. Early outcomes are good with very high survival. Most of the children are off invasive ventilation within first 24 hours and are discharged with in first week of surgery. Most children operated have good quality of life and survive into adulthood without re-interventions. Operated CHD patients can present in adulthood with issues of pregnancy, heart failure, arrhythmias and exercise intolerance and will need re- interventions or additional procedures.
Timing of intervention in CHD
Timing in CHD treatment is essential. Delay in treatment can unforgiving and result in irreversible pulmonary vascular changes and make child inoperable for life. Correct timing of surgery/ intervention is important and decided by the treating pediatric cardiac team balancing all the pros and Cons of early surgery. Decision on surgery should be made based on anatomy hemodynamics and severity of the lesion and not on basis of weight or comfort of surgeons and treating team. Broad guideline on when to intervene in different CHD.
- Obstructive lesion: Based on severity, usually as early as possible at any age.
- L->R shunts: Post tricuspid, from 6 weeks---3-6 months’ period
- Pre-tricuspid shunts: Usually before starting school/ Operable even in adulthood
- Critical CHD: Obstructed TAPVC, TGA, Truncus arteriosus, pulmonary atresia, As early as possible in neonatal period itself
- TOF physiology: Operable at any age/ Best to operate before 3 years as helps in lung development
Careful assessment of child/ baby is done and timing of intervention planned based on natural history, hemodynamics, severity of the lesion. There is no ideal weight for surgery and can be performed at any weight if lesion is severe and affecting growth of baby.
Care of child after cardiac surgery
Most children after cardiac surgery will need lifelong follow-up. Post-surgery most children/ babies are discharged to their homes. Parents are counselled and trained to take care at homes. Following are few guidelines for caring a child after cardiac surgery
- Feeding- breast feeding encouraged and complementary feeds after 6 months
- Vaccination as per schedule after 2 months after cardiac surgery
- Monitor for weight gain and development serially. Older children school performance can be assessed as a part of neurodevelopment.
- In case of RTI---Treatment with local child specialist at local hospital as per protocol
- Older children can go to school after 2months to avoid weight bearing till 6 months’ post-surgery. Daily moderate aerobic exercises after 2 months is recommended.
- General hand hygiene and avoid close contact / crowded place for 1-2 months post cardiac surgery
