Cyanotic Heart Disease;Overview of Management

ByDr. Ahmad Shaker

MD Cardiology

Definition

• Cyanotic Heart Disease is a defect or group of defects in the structure or function of the heart or the great vessels, present at birth, consisting of abnormal blood flow from the right to the left part of the circulatory system (either at the level of the atria, the ventricles, or the great vessels).

• This abnormal communication (called right-to-left shunt) results in poor oxygenation of the body and therefore cyanosis

Causes of Cyanotic Heart Disease:

• –Tetralogy of Fallot• –Transposition of the great arteries (D-TGA)• –Single ventricle• –Truncus arteriosus• –Total anomalous pulmonary venous

connections• –Ebstein’s anomaly• –Eisenmenger’s disease

Ductal Independent Mixing Lesions:

-Truncus Arteriosus .

-d-Transposition of Great Arteries.

-Total Anomalous Pulmonary Venous Connection without Obstruction (TAPVC).

Ductal-Dependent Pulmonary Blood flow:

-Tricuspid Atresia

-Pulmonic Atresia with Intact Ventricular Septum.

-Tetralogy of Fallot.

-Ebstein’s Anomaly.

Lesions with Ductal Dependent Systemic Blood Flow:

-Hypoplastic Left Heart Syndrome (HLHS).

-Interrupted Aortic Arch.

-Total Anomalous Pulmonary Venous Connection with Obstruction.

Increased Pulmonary Blood Flow

Decreased Pulmonary Blood Flow

Obstruction to Systemic

Blood Flow•Total Anomalous Pulmonary Venous Return•Truncus Arteriosis

•Tetralogy of Fallot•Transposition of the Great Arteries•Tricuspid Atresia

•Hypoplastic Left Heart Syndrome

Symptoms & Signs • Cyanosis.• Dyspnea.• Failure to thrive, or failure to grow properly• Fatigue • Squatting position after physical activity to

relieve breathlessness. • Hypoxic spells, characterized by: -Anxiety. -Hyperventilation. -Sudden increase in cyanosis.• Syncope.• Chest pain, Arrythmias. • CHF.

Diagnosis

Clinical:

-Upper left sternal border ejection murmur of RV outflow tract obstruction --------- TOF.

-Newborns present with severe cyanosis and a continuous murmur of ductal flow --------- pulmonary valve atresia and ductus arteriosus-dependent pulmonary blood flow .

-Present immediately after birth with severe cyanosis that progresses rapidly to metabolic acidosis------- TGA.

- Days to weeks after birth with heart failure and mild hypoxemia, A hyperdynamic precordium, wide pulse pressure, a normal S1 with a frequent ejection click, and a loud, usually single S2 are characteristic.------ persistant truncus.

- In persistent truncus, Heart murmurs vary and may include a flow murmur at the base, a loud regurgitant murmur at the lower left sternal border, and a mid-diastolic mitral flow murmur. With truncal valve insufficiency, a high-pitched diastolic murmur over the mid-sternum is present

ECG”

-Right axis deviation and RV hypertrophy on ECG ---- TFO.

-Combined ventricular hypertrophy ----- Truncus.

-Right axis deviation and Right Ventricular Hypertrophy ---- Total APVD.

-Superior axis and L V Hypertrophy ---- TV atresia.

-LV hypertrophy, leftward axis --- Pulmonic Atresia with Intact Ventricular Septum

-Right Bundle Branch Block, Delta Waves due to WPW syndrome --- Ebestine’s Anomaly.

TGA

Right ventricular hypertrophy, right axis deviation .

X-Ray:

TFO (Coure in Sabou).

- Small heart and a

concave main pulmonary artery segment with lung oligemia, Right aortic arch is present in 25%

- Cardiomegally with narrow base, lung hyperemia ---- TGA (Egg on side).

Anomalus Pulmonary Venous Drainage

Echocardiography

A Segmental Approach to cardiac Situs & malposition;

1- Atrial Situs:

Veseral Situs & visceroatrial concordance.

Atrial morphology (situs solitus or inversus).

venous inflow pattern.

2- Ventricular localization:

ventricular morphology.

atrioventricular concordance.

Base-to-apex axis (Levo or Dextrocardia).

3- Greate Artery Connections & its identifications.

4- Ventriculoarterial concordance.

Echocardiographic characteristics of Rt & Lt ventricles;

Rt Ventricle Lt Ventricle

*Trabiculated.*Three papillary.*Chorade insertes into ventricular septum.*Infundibular muscle band.*Moderator band.*Triangular cavity shape.*Tricusped valve with relatively apical isersion.

*Smooth.*Two papillary.*Ellipsoidal in shape.*Mitral valve with 2 leaflets & relatively basal insersion.

Tetralogy of Fallot

Ebstein’s Anomaly

Ebstein’s Anomaly

Tricusped Atresia

APVD

TGA

Truncus Arteriosus

Cathetrization

Tetralogy of Fallot

Trancus Arteriosus

Complications

• Heart failure and hypoxic spells.• Polycythemia and increased

coagulation.• Stroke.• Infective endocarditis.• Brain abscess.• Hemoptysis.• Impaired growth.• Pulmonary hypertension.

Prognosis

• Most children with congenital heart defects can be helped by surgery even if the defect is severe.   

•  Death rates attributable to congenital cardiovascular defects are only about two per 100,000, but they are considerably higher for infants under one year old.   

• For infants under one year, the death rate is about 60 per 100,000.

Treatment:

*‘tet’ spells: treatment• 1. ‘knee-chest position’ or over parent's shoulder with knees bent• 2. supplemental oxygen (effectiveness is questionable in the absence of pulmonary blood flow)• 3. sedation: intravenous or subcutaneous morphine, 0.1 mg/kg• 4. intravascular volume expansion• 5. prolonged cyanosis: an alpha agonist (phenylephrine, 5-10 mcg/kg IV)• 6. for prevention of spells: propranolol (0.5-1 mg/kg po

QID)

*The infants need to be monitored because of the resultant polycythemia, which may lead to hyperviscosity. Hyperviscous blood flows poorly through the circulatory bed and results in poor tissue perfusion.

*If blood flow to the systemic or pulmonic circulation is not sufficient to sustain life, prostaglandin E1 (PGE1) (0.05 to 0.1 µg/kg/min IV) can be administered to maintain patency of the ductus arteriosus.

*When a PGE1 infusion is being administered, blood pressure must be monitored and hypotension corrected.

Tetralogy of Fallot*Corrective operations are often performed by 18 months of age or earlier if the child has recurrent hypoxic episodes or progressive cyanosis.

*If pulmonary stenosis is severe, and supplemental blood flow through the ductus arteriosus is required to support oxygenation during the neonatal period.

*A palliative shunt is often placed, the most common of which is the Blalock-Taussig shunt. The shunting procedure involves anastomosis of the subclavian artery to the pulmonary artery, which will direct blood from the systemic circuit into the pulmonary bed and improve pulmonary blood flow.

*Corrective surgery involves closing the VSD with a patch, relieving the right ventricular outflow obstruction, and closing any previous palliative shunts.

*Early corrective surgery is preferable to a palliative procedure and can be performed in infancy if the pulmonary arteries are of sufficient size and the coronary artery connection is in a normal position.

*Without surgery, life expectancy is markedly reduced. Even with successful surgery, heart block, aneurysm formation, and late sudden death are possible.

Transposition of the Great Vessels*The arterial switch procedure is the surgical intervention of choice, since it returns blood flow to its normal pattern. This procedure involves cutting the great vessels above the valves and switching their positions with reimplantation of the coronary arteries.

*When corrective surgery is not possible, a palliative balloon or surgical septostomy is performed.

*If a balloon septostomy is not possible or not effective, a surgical septostomy (Blalock-Hanlon operation) can be performed.

*Corrective surgery involves partitioning the atrium and dividing the chamber into a front and a back section (Atrial Switch).

*Systemic venous blood is redirected in front of the partition toward the left ventricle and pulmonary venous blood is directed behind the partition toward the right ventricle.

*This partition can be made of a synthetic material (Mustard procedure) or of the child’s atrial septum (Senning procedure).

*The most common long-term complications of the Mustard and Senning procedures are arrhythmias

Truncus Arteriosus*Palliative pulmonary banding can be performed to improve the condition and development of pulmonary vascular disease until reparative procedures can be performed.

*Since banding increases mortality and complicates of corrective surgery, the corrective surgery is usually attempted in infancy. Correction involves closing the VSD so the left ventricle empties into the runcus.

*The pulmonary arteries are removed from the truncus and a conduit is inserted between the right ventricle and the pulmonary arteries (Rastelli procedure).

Total Anomalous Pulmonary Venous Return*Surgery is usually indicated soon after the diagnosis is confirmed. This operation involves the anastomosis of the pulmonary veins to the left atrium, closure of the ASD, and division of the anomalous connection.

*Without surgery, the prognosis is poor.

*Even if surgery is performed within days of birth, infants who have severe cyanosis and poor cardiac output before repair have high postoperative mortality. Connections above the diaphragm have a better prognosis than connections below the diaphragm.

Tricuspid Atresia*If pulmonary blood flow is minimal, palliative surgery is frequently necessary soon after birth. A connection is usually made between the subclavian artery and the pulmonary artery (Blalock-Taussig).

*Enlargement of the interatrial defect may be necessary to facilitate flow to the left atrium from the right.

*Corrective surgery usually involves a Fontan procedure, an anastomosis between the pulmonary artery and the right atrium. Any previous shunt procedure will be closed and the ASD patched.

Hypoplastic Left Heart Syndrome*Staged surgical repair of HLHS is still in its infancy and the mortality rate is high, but it does offer some hope for survival.

*The first surgery—the Norwood procedure—is performed during the neonatal period. A portion of the main pulmonary artery is anastomosed to the aorta, a shunt is performed to increase the pulmonary blood flow, and a large atrial septal defect is created.

*In the second stage, the volume load on the right ventricle is reduced.

*The final repair is a modified Fontan procedure, in which deoxygenated blood from the lower part of the body is directed to the right pulmonary artery shunt to mix with blood returning from the head and upper body and flow into the systemic circulation.

*Heart transplantation is also an option for infants with HLHS.

Thank You