27.2 Pediatric Cardiovascular & Collagen Disorders: Heart Sounds and Murmurs, Acyanotic and Cyanotic Congenital Heart Disease, Eisenmenger, Hypercyanotic Spells, Heart Failure, Rheumatic Fever, Hypertension, Endocarditis, Kawasaki, SLE, JIA, Dermatomyositis and Pediatric Resuscitation
Key Takeaways
Acyanotic left-to-right shunts (VSD, ASD, PDA) cause increased pulmonary blood flow and, if large and untreated, pulmonary hypertension with shunt reversal (Eisenmenger syndrome).
VSD gives a harsh holosystolic murmur at the left lower sternal border; ASD a fixed split S2; PDA a continuous "machinery" murmur.
Tetralogy of Fallot has a VSD, overriding aorta, pulmonary stenosis and RV hypertrophy; hypercyanotic spells are treated with knee-chest position, oxygen, morphine, fluids and propranolol.
Kawasaki disease needs fever for 5 days or more plus 4 of 5 criteria (conjunctivitis, oral changes, rash, extremity changes, cervical node); IVIG within 10 days with aspirin prevents coronary aneurysms.
In pediatric CPR, start with compressions (C-A-B) at 100 to 120 per minute and one-third of chest depth, with a 15:2 ratio when two rescuers are present.
Why this area matters
The TOS cardiovascular competencies are to:
- describe normal heart sounds and their sources;
- explain the hemodynamics of each disorder;
- arrive at a diagnosis and prioritize tests;
- recognize emergencies;
- plan drug and surgical treatment;
- educate the family, including about the cost of illness.
Congenital heart disease affects about 1% of live births. Rheumatic heart disease still affects Filipino schoolchildren, and Kawasaki disease is the leading cause of acquired heart disease in children in many countries. Surgery for congenital heart disease is expensive and limited to a few centers, so early diagnosis and timely referral matter.
Heart sounds and innocent murmurs
- S1: closure of the mitral and tricuspid valves.
- S2: closure of the aortic and pulmonary valves. Physiologic splitting widens on inspiration.
- A fixed split S2 suggests ASD.
- A single loud S2 suggests pulmonary hypertension, transposition or pulmonary atresia.
- S3 can be normal in children. S4 is always abnormal.
- Innocent (functional) murmurs:
- soft (grade 1 to 2), systolic, change with position, with no other findings;
- Still murmur (vibratory, left lower sternal border);
- pulmonary flow murmur;
- venous hum (continuous, disappears when lying flat or with pressure on the jugular vein).
- Pathologic features: diastolic or pansystolic murmurs, grade 3 or louder or with a thrill, abnormal S2, cyanosis, poor growth or signs of heart failure.
Congenital heart disease
| Lesion | Hemodynamics | Findings | Management |
|---|---|---|---|
| VSD (most common) | Left-to-right shunt; large defects cause heart failure at 6 to 8 weeks as pulmonary resistance falls | Harsh holosystolic murmur, left lower sternal border; small defects are loud | Many small muscular defects close; surgery for large defects with heart failure, failure to thrive or pulmonary hypertension |
| ASD (secundum) | Left-to-right at atrial level; RV volume overload | Wide, fixed split S2, soft systolic flow murmur at the upper left sternal border; often found late | Device or surgical closure in childhood |
| PDA | Aorta to pulmonary artery; common in preterms and congenital rubella | Continuous "machinery" murmur, bounding pulses, wide pulse pressure | Indomethacin, ibuprofen or paracetamol in preterms; device or surgical closure |
| Tetralogy of Fallot (most common cyanotic lesion after infancy) | VSD, overriding aorta, RV outflow (pulmonary) stenosis, RV hypertrophy; right-to-left shunt | Cyanosis, squatting, clubbing, systolic ejection murmur of pulmonary stenosis, boot-shaped heart with dark lung fields | Complete repair in infancy; Blalock-Taussig shunt as palliation |
| Transposition of the great arteries (most common cyanotic lesion in newborns) | Parallel circulations; survival depends on mixing (PFO, PDA, VSD) | Severe cyanosis in the first day, little murmur, "egg on a string" heart | Prostaglandin E1 to keep the ductus open, balloon atrial septostomy, then arterial switch |
| Coarctation of the aorta | Obstruction near the ductus | Weak or delayed femoral pulses, upper-limb hypertension; Turner syndrome | Repair; PGE1 in duct-dependent newborns |
- Eisenmenger syndrome: a long-standing large left-to-right shunt causes irreversible pulmonary vascular disease. The shunt then reverses, giving cyanosis, clubbing and polycythemia. Repair is no longer possible, so the aim is to close significant shunts early.
- Hypercyanotic ("tet") spells:
- sudden increased cyanosis, hyperpnea and irritability, often in the morning or after crying, feeding or defecation;
- infundibular spasm increases right-to-left shunting.
- Treatment:
- knee-chest position, which raises systemic vascular resistance;
- oxygen;
- morphine;
- IV fluids;
- sodium bicarbonate for acidosis;
- propranolol;
- phenylephrine if needed.
- Complications of cyanotic heart disease:
- polycythemia;
- brain abscess, usually after age 2, from bypass of pulmonary filtration, with headache, fever and focal signs;
- paradoxical embolic stroke;
- endocarditis.
Heart failure, endocarditis, hypertension and rheumatic fever
- Heart failure in infants:
- presents as poor feeding with sweating, tachypnea, poor weight gain, hepatomegaly and gallop;
- caused by large shunts and myocarditis;
- treated with diuretics (furosemide), ACE inhibitors, nutrition and correction of the lesion.
- Subacute bacterial endocarditis:
- viridans streptococci, especially after dental disease;
- prolonged fever, new or changing murmurs, splenomegaly and embolic phenomena;
- good dental hygiene and prophylaxis for the highest-risk lesions prevent it.
- Hypertension in children:
- BP at or above the 95th percentile for age, sex and height on three occasions; adolescents use adult cut-offs from age 13;
- younger children usually have secondary causes (renal parenchymal disease, renovascular disease, coarctation, endocrine), while adolescents with obesity often have primary hypertension.
- Acute rheumatic fever and RHD: the Jones criteria and secondary prophylaxis are covered in Microbiology. Carditis is the major determinant of long-term disability.
Collagen (connective tissue) disorders
- Kawasaki disease:
- a vasculitis of medium vessels, usually under age 5;
- fever of 5 days or more plus at least 4 of 5 features:
- bilateral non-exudative conjunctivitis;
- red cracked lips and strawberry tongue;
- polymorphous rash;
- swelling of the hands and feet with later periungual peeling;
- unilateral cervical node larger than 1.5 cm.
- Coronary artery aneurysms are the main risk.
- Treat with IVIG 2 g/kg within 10 days of fever onset plus aspirin, and follow with echocardiography.
- Juvenile idiopathic arthritis:
- arthritis for 6 weeks or more with onset before age 16;
- oligoarticular (4 or fewer joints; ANA-positive girls risk silent uveitis, so eye screening is needed);
- polyarticular;
- systemic (daily quotidian fever spikes, salmon-colored evanescent rash, serositis, risk of macrophage activation syndrome).
- Treat with NSAIDs, intra-articular steroids, methotrexate and biologics.
- Childhood SLE:
- more severe than adult SLE, with frequent lupus nephritis;
- malar rash, arthritis, cytopenias and serositis;
- ANA and anti-dsDNA with low complement;
- treat with hydroxychloroquine, steroids and immunosuppressants.
- Juvenile dermatomyositis:
- proximal muscle weakness (Gower sign);
- heliotrope rash of the eyelids and Gottron papules over the knuckles;
- raised CK;
- calcinosis.
- Unlike adults, there is no cancer association.
- Treat with steroids and methotrexate.
Pediatric resuscitation (the ABCs)
- Recognize arrest: unresponsive, not breathing normally and no pulse within 10 seconds. Shout for help and an AED.
- Compressions first (C-A-B):
- rate 100 to 120 per minute;
- depth one-third of the anteroposterior chest (about 4 cm in infants, 5 cm in children);
- allow full recoil and minimize interruptions;
- ratio 30:2 with one rescuer and 15:2 with two rescuers in infants and children.
- Techniques:
- two-thumb encircling technique for infants with two rescuers;
- heel of one or two hands for children;
- with an advanced airway, continuous compressions with 1 breath every 2 to 3 seconds.
- Drugs and shocks:
- epinephrine 0.01 mg/kg IV/IO every 3 to 5 minutes;
- defibrillation at 2 J/kg, then 4 J/kg for VF or pulseless VT.
- Remember: most pediatric arrests are respiratory or shock-related rather than primary cardiac. Early recognition of respiratory distress, failure and shock prevents them.
A 4-year-old boy with known congenital heart disease becomes intensely cyanotic and breathless after a prolonged crying episode. His mother says he often squats after running. A chest X-ray from a previous visit showed a boot-shaped heart with reduced lung markings. What is the first step in managing this episode?
Knee-chest position and oxygen, then morphine and IV fluids if it persists
IV furosemide to relieve pulmonary congestion as quickly as possible
IV digoxin to improve myocardial contractility during the spell
An IV prostaglandin E1 infusion to close the ductus arteriosus
A 3-year-old has had fever for 6 days with bilateral non-purulent conjunctivitis, red cracked lips, a strawberry tongue, a polymorphous rash and swollen red hands and feet. Which treatment most reduces the risk of the main complication?
Oral penicillin for 10 days for scarlet fever
Oral prednisone alone for 2 weeks
Supportive care alone, because the illness is self-limited and resolves within 2 weeks
IVIG 2 g/kg as a single infusion with aspirin, within 10 days of fever onset
A 2-month-old infant has poor feeding, sweating during feeds, tachypnea, poor weight gain and a liver edge 3 cm below the costal margin. A harsh holosystolic murmur is loudest at the left lower sternal border. Why did symptoms appear at this age rather than at birth?
The foramen ovale reopened as left atrial pressure fell
The ductus arteriosus closed in the first weeks, cutting off the extra pulmonary blood flow the heart depended on
Maternal antibodies that protected the heart disappeared
Pulmonary vascular resistance falls over the first weeks, increasing left-to-right flow through a large VSD
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