Cyanotic Heart Disease

Classification of Cyanotic Heart Disease

Cyanotic

Pulmonary blood flow Mixed blood flow

Tetralogy of fallot Transposition of great arteries

Tricuspid ateresia Total anomalous pulmonary Venous return truncus arteriosis Hypo plastic left heart

syndrome

Tetralogy of Fallot

Tetralogy of Fallot

• Introduction:Tetrology of fallot is the most common

type of cyanotic congenital heart disease.The classic form include four defect

• Ventricular septal defect• Pulmonary stenosis • Overriding aorta • Right ventricular hypertrophy

Pathophysiology The blood normally returns from the systemic

circulation to the systemic circulation to the

right atrium and right ventricles

The outflow of blood from the right ventricles is

resisted by the pulmonary stenosis so that

the blood flows through the ventricular septal

defect in to the aorta

There is right to left shunt. Hypertrophy of the

right ventricles occurs as a result of the

pressure exerted against the pulmonary

stenosis.

Because, the blood from the right ventricles is

unoxygenated, cyanosis results.

Clinical Manifestation

• Cyanosis, anoxia blue spells• Clubbing of the fingers and toes• Squatting posture • Slow weight gain• Dyspnoea no exertion • Hypoxic spells (low O2 in tissue)• Transient cerebral ischemia• Pan systolic murmur • Poor growth

Diagnostic Evaluation

• On Auscultation A loud harsh systolic murmur may be

heard at the left middle to lower sturnal border.

Radiography (X – Ray):

The heart shows the enlarged ventricle on the right side, the large aorta and the decrease in size of the pulmonary artery. The hilar areas of the lungs are usually clear because of normal or demised pulmonary blood flow. The unusual shape of the heart has led to its being called the wooden shoe or boot – shaped heart.

• ECG:

ECG shows evidence of right ventricular hypertrophy

• Echocardiography:

Shows evidence of the aortic override, thick, anterior right ventricular wall and large aorta.

• Cardiac catheterization:

• Angiocardiography

• Laboratory test – blood test

Management

• Oxygen administration for cyanosis

• Maintain nutrition by small and frequent

• Give rest and allow only minimum activities

for the child

Surgical Management • Palliative shunt:

In the infant who cannot undergo primary repair, a palliative procedure to increase pulmonary blood flow and increase O2 saturation may be performed. The preferred procedure is the modified blalock – taussing shunt, which provides blood flow to the pulmonary arteries from the left or right subclavion artery.

In general, however shunts are avoided because they may result in pulmonary artery distortion.

• Pott’s procedure :the upper decending aorta is anastomosed with left pulmonary artery

• Waterson shunt:it involves side to side anastomosis of ascnding aorta with right pulmonary artery.

• Brock’s procedure:pulmonary valvotomy done to correct PS

• Complete RepairElective repair is usually performed

in the first year of life. Indications for repair include increasing cyanosis and valves closure of the VSD and resection of the infundibular stenosis with a pericardial patch to enlarge the right ventricular outflow tract. The procedure requires a median sternotomy and the use of pulmonary bypass.

Tricuspid Atresia• Introduction:

Failure of the tricuspid valve to develop consequently no communication form right atrium to right ventricle. Blood flows through an atrial septal defect (ASD) or patent foramen ovale to the left side of the heart and through a VSD to the right ventricle and out to lungs.

• It is often associated with PS and transposition of the great arteries.

• There is complete mixing of unoxygenated and oxygenated blood in the left side of the heart.

• Resulting in systemic desaturation and varying amount of pulmonary obstruction causing decreased pulmonary blood flow usually is diminished.

Pathophysiology

At birth, the presence of a patent foramen ovale is required to permit blood flow across the septum into the left atrium.

CLINICAL MANIFESTATION

Cyanosis

Tachycardia

Dyspnoea

Chronic hypoxemia

with clubbing

Therapeutic Management

For the neonate whose pulmonary

blood flow depend on the patency of the

ductus arteriosus a continuous infusion of

prostaglandin E, is started until surgical can

be arranged.

Surgical Management

Palliative treatment is the placement of a shunt (systemic to pulmonary artery) to increase blood flow to the lungs. If the ASD is small, an atrial septostomy is done during cardiac catheterization some children have increased pulmonary volume of blood to the lungs. A bidirectional glannshunt may be performed at 6 to 9 months as a second stage.

MODIFIED FONTAN PROCEDURE Systemic venous return is directed

to the lungs without a ventricular pump through surgical connections between the right atrium and the pulmonary artery. A fenestration (opening) in the right arterial baffle is sometimes done to relieve pressure. Patient must have normal ventricular function and a low pulmonary vascular resistance for the procedure to be successful.

The modified fontan procedure separates

oxygenated and unoxygenated blood inside the

heart and eliminates the excess volumes load

on the ventricle but does not restore normal

anatomy or hemodynamic.

Complication

Dysrhythmias

Systemic venous hypertension

Pericardial effusion

TRANSPOSITION OF THE GREAT ARTERIES

Transposition of great vessels (TGA) is

defined as aorta arising from the right ventricle and pulmonary artery from the left ventricle.

Sign & Symptoms

Cyanosis

Congestive heart failure

Fatigue

Slow weight gain

Clubbing of the finger

Rapid breathing

Heart size enlarge rapidly

Pathophysiology

In this anomaly the aorta has its origin in the right ventricles and pulmonary artery has its origins in the left ventricles.

Hence, the aorta carries unoxygenated blood to the systemic circulation and the pulmonary circuit carries oxygenated blood back to the

lungs.  

The pulmonary venous return is to the left atrium and the systemic veins returns to the

right atrium.

There is two separate circulatory systemic exist, one pulmonary and one systemic. An infant can survive with this malformation initially only if an associated with defect or PDA

is present 

There co-existing lesions provide a means for mixing venous and

arterial blood.

Diagnostic Evaluation

X – RayE.C.GCardiac catheterizationAngiocardiographyBlood examinationEchocardiogram

Treatment The medical management consist in control

of congestive failure. PALLIATIVE OPERATION

• Blalock Hanlon procedure -surgical creation or enlargement of an existing arterial septal defect.

• Rash kind operation – enlargement of an existing arterial septal defect.

CORRECTIVE SURGERY Mustard’s operation – the complete repair of

the defect is done. 

Nursing Management• Provide comfort and adequate rest to the

child• Avoid excessive crying.• Observe the clinical features make the

nursing diagnosis & plan nursing action to be carried out.

• Report the unusual symptoms like sign of cardiac failure

• make proper positioning of the child• Oxygen inhalation is given for cyanosis• Diuretic to reduce over hydration

• Maintained the fluid & electrolyte balance • Blood test should do to check acidosis.• Small frequent feeds should be given• Provide psychological support• Provide love & affection to the child• Meet all his hygienic needs with the help

of mother.• administration of proper antibiotics• Watch the possible complication