Cardiology
Section 6: Cardiology
6.2 HEART DISEASES SUBSECTIONS .1 X-rays
6.2.3 Echocardiography
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Absent Pulmonary Valve Syndrome
Figure 6.2.3.3: Absent pulmonary valve syndrome
Photo Courtesy: M Zulfikar Ahamed
Absent pulmonary valve syndrome is a rare variant of TOF. It has characteristically, rudimentary pulmonary valve and dilated PA and branches. It can present in the newborn with cyanosis, RDS, stridor and a loud systolodiastolic murmur.
The picture shows the presence of PS and PR, both in color and continuous wave (CW) Doppler.
The course in the newborn may be stormy. Maximum mortality occurs in newborn period due to CHF, respiratory distress and hypoxemia.
Once the neonatal period is over, the baby stabilizes usually and is a candidate for intracardiac repair (ICR) with transannular patch.
Coronary Artery Dilatation in Kawasaki Disease
Figures 6.2.3.4A and B: Coronary artery dilatation in KD
Photo Courtesy: M Zulfikar Ahamed
Both frames show coronary artery dilatation (CAL) of left anterior descending artery in Kawasaki disease. CAL can be classified as mild (<4 mm) , moderate (4–8 mm) and giant (>8 mm). Fifty percent of CAL regress in one year. Giant aneurisms do not usually regress.
Low dose aspirin is given indefinitely (5 mg/kg). Larger aneurisms may require addition of clopidogrel (1 mg/kg) along with low dose aspirin. Giant aneurism may be managed with oral
anticoagulant to keep INR between 1.5 and 2.
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Lutembacher Syndrome
Figures 6.2.3.7A and B: Lutembacher syndrome Photo Courtesy: S Harikrishnan
The upper panel shows rheumatic MS with moderate ostium
secundum ASD. In the lower panel color jet delineates both ASD and MS. Lutembacher is extremely rare.
One reason for ASD with a loud murmur is Lutembacher. MS can worsen symptoms of ASD and ASD can mitigate the hemodynamic effects of MS like PVH.
Management is essentially surgical.
Balloon mitral valvotomy (BMV) for MS and device closure for ASD can be attempted.
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Ebstein Anomaly
Figure 6.2.3.6: Ebstein anomaly Photo Courtesy: M Zulfikar Ahamed
The apical view shows LA and LV on the left side. RA with anterior tricuspid leaflet is seen. There is a distal displacement of septal leaflet producing an atrialized RV (ARV).
The true RV is relatively small.
Ebstein anomaly is quite rare (0.5%), but is a fascinating CHD. It can present with shock, CHF, cyanosis or all in the newborn period. Twenty percent of Ebstein can have WPW syndrome.
The treatment of significant Ebstein is surgery—TV valve repair, plication of atrialized RV and closure of the ASD. Indications of this sort of surgery (Danielson’s repair) are class III and IV status, deepening cyanosis, progressive cardiomegaly and refractory SVT.
Device Closure of ASD
Figure 6.2.3.5: Device closure of ASD Photo Courtesy: S Sivasankaran
Top film shows placement of the device for ASD closure. The bottom is a 3D echo of an ASD closed by device.
Sixty percent of OS ASDs are closed by device. Ostium primum ASD and sinus venosus ASD are closed by surgery only.
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Mitral Stenosis (MS)
Figures 6.2.3.8A to C: Mitral stenosis Photo Courtesy: M Zulfikar Ahamed
The upper panel (PS LAX) shows the doming, thick mitral valve with a large LA. The bottom panels show the narrow mitral valve opening (very much small mitral valve area) Doppler interrogation of mitral valve indicating severe MS. Mitral valve area can be calculated by pressure half time method along with 2 D measure. Normal MV area is 4 cm2/M2. MS is said to exist when MVA is < 2.5 cm2. Severe MS in the young (< 20 years) is called juvenile MS. It is almost always rheumatic.
Congenital MS can occur rarely.
Initial management is medical—
rest, diuretic and b-blockers. The standard treatment of significant MS is balloon mitral valvotomy.
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Ostium Secundum ASD with L→ R Flow
Figure 6.2.3.10: Ostium secundum ASD with L → R flow
Photo Courtesy: M Zulfikar Ahamed
The echo picture is a four chamber view showing a reasonably sized Ostium Secundum ASD with L→R flow. There is evidently RV volume overload. ASD forms 10% of all CHD. The other types of ASD are primum and sinus venosus.
All OS ASD except small (shunt
<1.5 : 1) should be closed. Sixty percent of ASDs can be closed by device and rest by surgery. Surgical mortality is near zero. Thirty years survival if surgery is done before 11 years is near control population.
Mitral Regurgitation—Rheumatic
Figure 6.2.3.9: MR—Rheumatic Photo Courtesy: M Zulfikar Ahamed
The first panel shows
morphoanatomy of rheumatic MR. Other frames indicate varying degrees of MR by color—from trivial to mild to moderately severe.
All rheumatic MR are given rheumatic prophylaxis as well as endocarditis prophylaxis. No other drug is indicated for mild-moderate MR. Moderate-severe MR may require ACE inhibitors. Surgery for severe MR in children is preferably mitral valvuloplasty.
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Rhabdomyoma in the LV
Figure 6.2.3.12: Rhabdomyoma in the LV Photo Courtesy: M Zulfikar Ahamed
Rhabdomyoma are the most common benign tumors of the heart in children. They are pedunculated masses which are usually found in ventricles, while myxomas are found in atria. Rhabdomyoma can be asymptomatic and may regress.
It can also produce LVOT/RVOT obstruction, CHF and ventricular arrhythmia. This is associated with tuberous sclerosis.
Treatment is conservative. Large persisting ones in RV are surgically removed. LV rhabdomyoma are usually left untouched as it may involve left ventriculotomy and are of high-risk.
PDA with L→R Shunt
Figure 6.2.3.11: PDA with L→R shunt Photo Courtesy: M Zulfikar Ahamed
PDA flow is picked up at pulmonary artery by color Doppler. The
continuous flow signal on the left hand side indicates the gradient across PDA between aorta and pulmonary artery in systole and diastole. It is called aorto pulmonary gradient. From this value,
approximate PA pressures can be calculated.
All PDAs except silent, trivial PDA are to be closed. Practically all of them are closed by a coil (small
<3.5 mm) or device (moderate and large). Surgery is seldom required.
The age of closure may vary depending on the size of the shunt and symptoms.
Tetralogy of Fallot
Figures 6.2.3.13A and B: Tetralogy of fallot Photo Courtesy: M Zulfikar Ahamed
The echo picture demonstrates (on parasternal long axis) a large subaortic VSD with override of aorta. The apical view clearly shows the large, malaligned VSD and nearly 50% aortic override. Aorta appears to arise from both LV and RV. Right ventricular outflow tract (RVOT) obstruction is to be assessed in parasternal short axis view and is not shown here. TOF is the most common cyanotic CHD in infants and children and accounts for 10 to 15% of all CHD.
The management of choice in TOF is intracardiac repair around 1 year of life. If the baby is severely cyanosed or has frequent spells early in life, one needs to do a BT shunt as a palliative measure.
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Viral Myocarditis
Figure 6.2.3.16: Dilated cardiomyopathy Photo Courtesy: M Zulfikar Ahamed
2 D picture shows a grossly dilated LV with thin walls. There is globularity of LV with LV enlargement. In real time, the contractility will be poor and there will be significant MR.
Standard management of viral myocarditis includes use of IV inotropes, ACE inhibitors, digoxin and diuretics. IVIG could be useful in children with myocarditis.
Vegetations on Aortic Valve
Figure 6.2.3.15: Vegetation on aortic valve Photo Courtesy: S Sivasankaran
This is a parasternal long axis view (LAX) showing echodense nodule on aortic valve. Mitral valve is normal. In real time the vegetations are freely mobile. Infective
endocarditis (IE) of the aortic valve usually occurs on bicuspid aortic valve or rheumatic aortic valve disease.
Infective endocarditis has a fairly high mortality - 30%. It needs aggressive antimicrobial treatment at least for 4 weeks. The usual organisms are S. viridans and S. aureus.
Unobstructed TAPVC
Figure 6.2.3.14: Unobstructed TAPVC Photo Courtesy: M Zulfikar Ahamed
Observe the very large RA and RV and the diminutive LV. LA is small and there is a posterior chamber into which pulmonary veins drain.
Unobstructed supracardiac TAPVC presents in the newborn and early infancy with severe CHF and mild cyanosis. This will present like an ASD with ‘cyanosis’. TAPVCs are classified into infracardiac, cardiac and supracardiac.
Management consists of early stabilization with inotropes, diuretics, oxygen and urgent surgical repair. Mortality is around 5 to 10%. However, the survivor will have a near normal life.
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Balloon Mitral Valvotomy (BMV)
Figure 6.2.4.3: Balloon mitral valvotomy Photo Courtesy: S Harikrishnan
Angio pictures of progressive dilatation of stenosed mitral valve orifice. The classical dumbbell appearance of the balloon across mitral valve is also seen.
BMV is the procedure of choice in MS in all ages. It has supplanted CMV. The valve area doubles with a minimal risk of MR and the good result lasts for at least 10 years.
Figure 6.2.4.1: Catheterization lab Photo Courtesy: M Zulfikar Ahamed
The photograph shows a diagnostic catheterization lab.
• Diagnostic catheterization in CHD has given way to catheter interventions. Almost all PDAs, more than half of ASDs, a small percent of VSDs and a few AP windows are closed by device. Balloon valvotomy is the treatment of choice in PS, AS, MS and TS. A significant proportion of CoA is managed with Balloon and stent.
• Other uses of catheterization are stenting of PDA, closure of CAVF, MAPCA, Balloon septostomy, etc.
Catheterization Lab
Balloon Dilatation of Pulmonary Valve (BPV)
Figure 6.2.4.2: Balloon dilatation of pulmonary valve
Photo Courtesy: S Sivasankaran
Angio picture shows the process of dilatation of stenotic pulmonary valve by a balloon. The waist is seen, which will be completely abolished once successful dilatation is over.
BPV is quite safe and offers an excellent result in >95% and the result is long lasting. The mortality is near zero. BPV is offered when PS gradient is more than 50 mm Hg.