Congenital Heart Disease

MEDICINE II Congenital Heart Disease Bernardo D. Morante Jr. MD 2nd Shifting – 2008 Sept 17 The black swindler

Normal Cardiovascular system • The right side and the left side of the heart are anatomically and functionally separated by the interatrial and interventricular septa. • There are no connections between major arteries and veins.

• Factors • • • • •

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Only capillary connections systemic circulation exist.

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contributing to Congenital cardiac anomalies Drugs ex. Thalidomide, Dilantin, Lithium Alcohol and illegal drugs Chromosomal abnormalities ex. Trisomy 21 (Down’s syndrome) Infections during pregnancy such as Rubella Connective tissue disorders Inborn errors of metabolism Family history of congenital anomalies

They are anomalous connections between the right side and left side of the heart or between the venous and arterial circulation

Direction of Shunts Since pressures in the left side of the heart are usually higher than the right side, in shunts the blood flows from left to right. However right to left shunts occur when the following conditions are present: • Severe pulmonary hypertension • Right ventricular outflow obstruction as in Tetralogy of Fallot

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Obstruction to the inflow of blood as in tricuspid atresia Pulmonary arteriovenous fistula Anomalous origin of the major arteries ATRIAL SEPTAL DEFECTS

Sinus venosus Ostium secundum Inferior vena caval Ostium Primum

Shunts

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The severity of the congenital defect is measured by the degree of shunting of the blood, the Qp/Qs ratio.

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Qp = the pulmonary blood flow Qs = the systemic blood flow

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Shunts • What are shunts? •

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Types of congenital cardiac anomalies

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With shunt a. Right to left shunt _cyanotic arterial hypoxemia is present b. Left to right shunt_ acyanotic step up in O2 content on the right side of the heart

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Without shunts

Acyanotic congenital anomalies (L-R shunt) 1. Intracardiac shunts a. atrial septal defect (ASD) b. ventricular septal defect (VSD)

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Extracardiac shunts a. Patent ductus arteriosus (PDA) b. Peripheral arteriovenous fistulas c. Partial anomalous venous return

Congenital Cardiac Anomalies without shunts 1. Valvular anomalies 2. Ebstein anomaly* 3. Cor tri-atriatum* 4. Coarctation of the Aorta 5. Idiopathic pulmonary hypertension 6. Cardiomyopathy 7. Dextrocardia *Maybe associated with shunts The pulmonary circulation in congenital heart disease 1. In left to right shunts the arterial vascularity is increased 2. In right to left shunts arterial vascularity is diminished 3. In congenital anomalies without shunts the arterial vascularity is normal

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When congestive heart failure occurs there is an increase in venous vascularity.

Atrial Septal Defects Types: 1. Patent foramen ovale 2. Ostium secundum 3. Sinus venosus 4. Inferior vena caval 5. Ostium primum

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Single atrium

Symptoms of acyanotic congenital heart diseases • Easifatigability • Dyspnea MARY YVETTE ALLAIN TINA RALPH SHERYL BART HEINRICH PIPOY KC JAM CECILLE DENESSE VINCE HOOPS CES XTIAN LAINEY RIZ KIX EZRA GOLDIE BUFF MONA AM MAAN ADI KC PENG KARLA ALPHE AARON KYTH ANNE EISA KRING CANDY ISAY MARCO JOSHUA FARS RAIN JASSIE MIKA SHAR ERIKA MACKY VIKI JOAN PREI KATE BAM AMS HANNAH MEMAY PAU RACHE ESTHER JOEL GLENN TONI

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Palpitations/ syncope Frequent respiratory infections Exertional cyanosis may occur when shunts are large

When the left to right shunt is small _ Qp/Qs ratio of 1.5 or less, patient is usually aymptomatic. SECUNDUM ATRIAL SEPTAL DEFECT KEY PE findings: • SEM at the pulmonic area that diminishes with Valsalva maneuver • Wide fixed splitting of S2 • If the left to right shunt is big there will be a diastolic flow murmur at the tricuspid area Diagnostics in Secundum ASD EKG: • Incomplete RBBB _ rsR pattern • Right axis deviation Chest X-ray: • Right heart border will be prominent suggesting RA enlargement • Obliteration of the retrosternal space in the lateral view due to RV dilatation

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Hilar blood vessels will be enlarged with prominence of the MPA. The arterial vessels will extend peripherally Echocardiogram in Secundum ASD • Dilated right ventricle with paradoxical septal motion • Drop out of echoes in the septal region (septal defect) in the subxyphoid view • Blood flow from LA to RA through the shunt will be observe on the doppler study • On air contrast study a filling defect will be observed at the site of the septal defect Cardiac Catheterization in secundum ASD • Catheter can be passed through the septal defect • Step up in O2 content or saturation at the atrial level • Qp/Qs ratio of 1.5 or greater. Therapy • Medical treatment for CHF • Control arrhythmias • Transcatheter closure of the ASD

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Surgical repair

OSTIUM PRIMUM DEFECT or ENDOCARDIAL CUSHION DEFECT Pathology: • a septal defect low in the atrium • Cleft mitral or tricuspid valve is present

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a VSD may also be present (common AV canal). • CHF appear early • More rapid development of pulmonary hypertension • Associated with Down’s syndrome Key PE findings • Pansystolic murmur at LSB representing the VSD • SEM at the pulmonic area • A holosystolic murmur of MR at the apex which radiates to the axilla

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A blowing systolic murmur of TR at the lower LSB which increases on inspiration Diagnostics in ostium primum defect EKG: • Incomplete RBBB/ occasionally complete RBBB • Left axis deviation

• 1st degree AV block Chest x-ray: 1. LV enlargement 2. Bi-atrial enlargement

Echocardiography in ostium primum defect • Presence of atrial septal defect low in the atrium with or without VSD • Cleft mitral / tricuspid valve

• Blood flow from LA to RA on the doppler study Cardiac catheterization in ostium primum defect • Step up in O2 content or saturation at the atrial and ventricular level • On angiography, flow from LV to RV is noted LV angiogram in ostium primum defect Note: may moving diagram sa slide Therapy • Treatment of CHF • Control arrhythmias •

Surgical repair

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VENTRICULAR SEPTAL DEFECT Is a cardiac anomaly where part interventricular septum is absent.

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Types: • VSD over the crista supraventricularis • VSD below the crista supraventricularis • At the muscular interventricular septum Pathophysiology of VSD

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If the defect is < 1 cm2 it is usually well tolerated Spontaneous closure may occur Defects greater than 2 cm2 results in biventricular volume overload

• Large VSD develops Eisenmenger syndrome early Key PE findings in VSD • A thrill at LSB with a harsh holosystolic murmur • Wide splitting of S2 that varies with respiration •

S3 at the apex

Diagnostics in VSD EKG: • Maybe normal in small VSD • Presence of LVH, LAH, left axis deviation Chest x-ray: • Normal in small VSD • RV and LV enlargement • MPA enlarged with plethora of the pulmonary vessels Echocardiogram in VSD • The defect in the interventricular septum can be visualized

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In the color doppler, there is a left to right flow across the interventricular septum Cardiac catheterization in VSD • Step up in O2 content or O2 saturation in the RV • On LV angiography, x-ray contrast crosses to the right ventricle Therapy: Surgical closure PATENT DUCTUS ARTERIOSUS Definition: It is the persistence of the ductus arteriosus beyond twenty days in infancy Types of PDA 1. PDA whose pulmonary pressures never return to normal

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PDA whose pressure return to normal in infancy but the pulmonary pressures rises as the child grows.

Key PE finding in PDA

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A thrill and a continuous “machinery” like murmur at the upper LSB or left infraclavicular region.

Diagnostics in PDA EKG: LVH and LAH Chest x-ray:

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Cardiomegaly with prominence of the apex suggesting LV dilatation Echocardiogram in PDA • Dilated main pulmonary artery • Left ventricular volume overload • On the doppler, blood flow will be seen from the aorta to the pulmonary artery Cardiac catheterization in PDA • There is a step up in O2 saturation in the pulmonary artery

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Surgical ligation of the PDA EISENMENGER SYNDROME

SECONDARY PULMONARY HYPERTERNSION • In large left to right shunts, secondary pulmonary hypertension eventually develops. • When the pulmonary pressures equals or exceeds systemic arterial pressure, this is known as Eisenmenger syndrome.

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Bi-directional shunting usually exist

PE findings in Eisenmenger syndrome • Visible or palpable pulmonary pulsation • Sternal lifting or subxyphoid pulsation suggesting RVH • P2 is increased, narrowed splitting or single S2 • The systolic murmurs of ASD and VSD will diminish • The continuous murmur of PDA may become a systolic murmur only • TR and PR murmurs (Graham Steele) will be heard.

Peripheral cyanosis

Diagnostics in Eisenmenger syndrome EKG: RVH, RAH, right axis deviation Chest x-ray: • Prominent right heart border • Obliteration of the retrosternal space • Prominent MPA and hilar vessels but the peripheral vessels will diminished Echocardiogram: • RVH and RAH • Tricuspid regurg and pulmonic regurg on the doppler • Very high pulmonary pressures Implications on the therapy of congenital anomalies • When severe pulmonary hypertension develops, surgical correction of the congenital anomaly is no longer feasible. • Most vasodilators are not effective in lowering pulmonary pressures • The following have been shown to benefit patients: 1. Calcium channel blocker_ Nifedipine 2. Prostacyclin _ epoprostenol 3. Endothelin receptor antagonist_ bosentan 4. sildenafil (Viagra) • Heart-lung transplant •

CONGENITAL ARTERIO-VENOUS FISTULA It is an abnormal communication between a large peripheral artery and vein.

Common sites: 1. Lower extremity at the ilio-femoral region 2. Hemangiomas in the liver and lungs (Hereditary telangiectasia)

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On aortic angiography, x-ray contrast flows from the aorta to the pulmonary artery

Therapy of PDA • Prostaglandin • Transcatheter closure of the PDA

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If the shunt is small, patient is asymptomatic. Otherwise signs of high output failure maybe present.

PE findings in AV fistulas • Systolic hypertension and wide pulse pressure • tachycardia • Systolic pulmonic flow murmur • Erythema and warmth in the involved extremity • Presence of a thrill and continuous murmur at the site of the fistula. • Branham’s sign: on compression of the fistula the heart rate will drop • Quinke’s sign Diagnostics:

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EKG: sinus tachycardia, LVH Chest x-ray: Cardiomegaly Echocardiogram: bi-ventricular dilatation

Therapy: Ligation of the fistula COARCTATION OF THE AORTA •

It is a constriction in the aorta usually after the origin of left subclavian artery

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It presents as hypertension. The symptoms and findings depends on the severity of the coarctation and site.

Pathophysiology in coarctation of the aorta • The hypertension is due to the gradient across the coarctation

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And the response aldosterone system

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Symptoms of Coarctation of the aorta • Headaches • Leg fatigue and intermittent claudication

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Signs of CHF due to uncontrolled hypertension

PE findings in coarctation of the aorta • Hypertension with differential BP between the upper and lower extremity • Thrills at the supra sternal notch • Collateral vessels in the scapula • Systolic ejection murmur in the interscapular region

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A systolic murmur is heard in the 2nd RSB if a concomitant bicuspid aortic valve is present

Diagnostics in coarctation of the aorta EKG : LVH Chest x-ray: • the 3 sign _ dilated aorta and cardiomegaly • Rib notching posteriorly

Echocardiogram: • The coarctation maybe visualized depending on the site. • The pressure gradient can be measured by doppler. CT scan or MRI angiogram: the presence and the site of the coarctation is confirmed. Complications • CHF • Aortic rupture • Bacterial endocarditis

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CVA due to rupture of Berry aneurysm

Therapy • Antihypertensive therapy • Treatment of CHF • Balloon dilation • Surgery TETRALOGY OF FALLOT Pathological findings: • VSD • Infundibular stenosis • Over riding of the aorta • RVH EIBSTEIN ANOMALY

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