Cynotic congenital heart disease

Congenital heart diseases

Coarctation of aorta

Constriction that occur at any portion of aorta from transverse arch to the iliac bifurication.more in male afeatures of turner syn

Pathophysiology :

2 types
Juxtaductal ( if mild adult type) 98% just below origin of lt subclavian art.
Tubular hypoplastic ( infantile type ) .

If more severe juxtaductal or tubularhypoplastic blood pass through ductus arteriosus decsending aorta if closed diffrential cyanosis ( lower blue extremities and upper pink extremities ).






In COA:
blood pressure in area proximal to coarctation
( mechanical and hormonal )
blood pressure distal to the coarctation.
Development of collaterals from subcalvian , internal mamery , superior intercostal .

Clincal features

If mild and Dx after infancy rarely significant symptoms and most diagnosed by blood pressure with routine physical examination.
Signs :
Pulses of UL and LL.
Radio-femoral delay.
Blood pressure in both UL and LL.
Blood pressure in each arm.
Ejection systolic click + thrill.
Systolic murmur in 3rd , 4th Lt sternal border .
Mid-diastolic murmur.
Systolic murmur of aortic stenosis

In neonatal period

Lt body hypoperfusion, acidosis, HF .
Before ductal closure differential cynosis.


Diagnosis
• CXR
• ECG
• ECHO
• COLOR DOPPLER
• CONTINOUS AND PULSED WAVE DOPPLER
• CATHETRAIZATION + LT VENTRICULOGRAPHY
• MRI used in dx due to difficulty in infancy

Complications

• Untreated succumb at 20-40 yr.
• Complications secondary to
• IE, Endarteritis
• Aneuryzms of the descending aorta or collaterals.
• Neonates : hypoperfusion + HF
• Premature coronary arterey disease
• HF
• ICH
• Hypertensive encephalopathy

Treatment

• In neonate PGE1 reopen ductus and relieve obstruction stabilize him surgery.
• Older children HF antifailure surgery
• Surgery:
Excision and primary re-anastamosis
Subclavian flap.
Patch aortoplasty.

Pulmonary stenosis

Pulmonary stenosis divided anatomically into :
• Vulvular ( most common ) .90%
• Supravalvular .
• Subvalvular.

Pathophysiology

Obstruction Rt. Ventricular pressure during systole wall stress severe cases RVH.
Pulmonary art. Pressure normal or .
Arterial O2 normal unless VSD or ASD.
Critical pulmonic stenosis in neonate shunt at the foramen ovale

Clinical features

According to severity

• Mild : asymptomatic , normal venous pressure , ejection click after 1st heart sound , 2nd heart sounds split , short ejection systolic murmur in pulmornary area .
ECG: normal or mild RVH.
CXR: poststenotic pulmonary arterial dilatation.
ECHO: pr.gradient usually 10-30mmHg

• Moderate : slightly elevated venous pr. , prominent a – wave in jugular pulse, 2nd heart sound split , ejection click, ejection systolic murmur
• CXR: normal or pulm.vascularity .
• ECG: RVH, spiked p-wave.
• ECHO: thickened valve , pr.gradient 30-60 mmHg.
• .

• Severe: Rt.sided failure, hepatic enlargment, periphral edema , venous pr. a-wave , heart enlarged , inaudible pulmonary component of 2nd heart sound , ejection systolic murmur and thrill , no click.
CXR: enlarged heart + pulmonary vascularity.
ECG: RVH, spiked p-wave.
ECHO: valve deformity, RVH.,pr.gradient ≥ 60 mmHg

Treatment

• Mild reassurance .
• Moderate or severe balloon valvuloplasty .
• Critical pulmonic stenosis valvuloplasy or surgery.valvutomy

Tetralogy of Fallot

It is the commonest cyanotic heart disease in children , it’s a combination of :-

• Right ventricular outflow obstruction(pulmonary stenosis)
• VSD
• Dextroposition of aorta with override of ventricular septum.
• Right ventricular hypertrophy.

RV outflow obstruction : various sites , but most common is infandibular site .

VSD large , non restrictive .

Overriding of aorta ( right sided in 20% ).

Clinical features

Time of onset of symptoms

Severity of cyanosis
RV hypertrophy

Depend of the degree of right ventricular outflow obstruction

If mild initially heart failure with age , patient grows infandibular hypertrophy cyanosis develops in 1st year of life .

If severe obstruction cyanosis develops immediately after birth .

In older children , long standing dusky blue skin, grey sclera, engorged blood vessels, clubing of fingers & extracardiac manifestation.
Dyspnea on exertion so they stop to take rest or have squatting position.
Growth retardation ( if severe and untreated ) .
Delayed puberty .

O/E

Pulse normal
Venous and arterial pressure normal .
Heart size normal .
Lt. hemithorax bulged because of RVH.
Murmur , ejection systolic because of RV outflow obstruction .
Murmur can be holosystolic due to VSD.
The intensity of murmur during spells .
Sometimes continuous murmur due to collaterals.

Diagnosis

• CXR : boat shaped heart , clear lung field , 20 % right sided aorta .
• ECG : RVH , right axis deviation .
• ECHO
• Cardiac cathetarization .
• Selective right ventriculography : important for child as surgical candidate .
• Lt. ventriculography .
• Coronary angiography .

Complications

• Cyanotic spells
• Cerebral thrombosis
• Brain abcess
• Infective endocarditis
• Heart failure

Cyanotic spell

Usually at 4-6 mo.
Patient restless , cyanosed , gasping , syncop follows .
Mainly after awakening or vigorous exercise .
intensity of the murmur.
Continued for few min.-few hrs .
Followed by generalized weakness , sleep.

Treatment of spell

• Put him on abdomen , knee-chest position.
• O2 .
• Morphine ( 0.2mg/kg s.c ) relaxe pulmonary infandibulum and sedate child .
• If severe NaHCO3 to correct acidosis .
• If still resistant phenylephrine or methoxantheme to systemic vascular resistance and Rt. Lt shunt .


Treatment
Medical :
• If severe obstruction medical Rx until surgical intervention.
• Include
• Provide O2 , maintain body temperature .
• Treat and prevent hypoglycemia .
• Start PGE1 infusion
• If less severe obstruction and while await for the surgery observe for the following:

Rx dehydration.

Rx iron deficiency anemia .
Inderal 0.5-1 mg/kg 6hr.
Phlebotomy if symptomatic patient and hematocrite > 65%.

Surgical Rx.

2 options : palliative
corrective

Time : 4-12 mo.

Palliative surgery

Modified Blalock – Taussing shunt , which a conduit from subclavian artery to homolateral branch of pulmonary art. or directly from ascending art. To main pulmonary artery .

.





With increasing age need for more pulmonary blood flow do corrective surgery or reanastomose on the opposite site

Transposition of great vessels

CCHD with pulmonary blood flow .
Either d-TGA or l-TGA.
Either with intact VS or VSD.
Male > female .
50% with VSD .

For d- TGA aorta arises from RV and pulmonary art. From LV .

Aorta anterior and to the right of pulmonary art.

C/F: cyanosis & tachycardia .

HF less common
if untreated not survive neonatal period .





Dx
ECG Rt. Sided dominance pattern.
CXR mild cardiomegaly.
Hyperoxia test.
ECHO.
CATHETRIZATION.

Treatment

Infusion of PGE1
Protect against hypothermia , Rx acidosis & hypoglycemia .
If no response Rashkind Balloon septostomy then arterial switch operation ( Jantene operation ) within 2 wks.
If TGA&VSD do Rashkind operation.

Extracardiac manifestation of CCHD.

• Polycythemia .
• Relative anemia .
• CNS abcess.
• CNS thromboembolic stroke.
• Low grade DIC , thrombocytopenia.
• Hemoptysis .
• Gum disease .
• Gout .
• Arthropathy , COF.
• Infection .
• Pregnancy complications .
• FTT.
• Psychosicial problems .

Thank you