Cong heart diseasea

CONGENITAL HEART DISEASES

Soumya Ranjan ParidaBasic B.Sc. Nursing 4th year

Sum Nursing College

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Practical approach Congenital-Associated syndrome Acquired

Rheumatic Cardiomyopathy ( Restrictive ) Cardiomyopathy ( Dilated , Post viral

myocarditis ) Pericardial diseases ( Effusion,Pericarditis) Kawasaki disease

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CLINICAL CLASSIFICATION OF CONGENITAL HEART DISEASES

Cardiac Malpositions- Ectopia cordis Dextrocardia

Acyanotic without a shunt– Malformations on left side Malformations on right side

Acyanotic with a shunt Cyanotic

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Acyanotic without a shunt Left sided malformations

Mitral stenosisMitral regurgitationAortic stenosisAortic regurgitationCoarctation of aorta

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Acyanotic without a shunt Right sided malformation

Ebsteins anomaly of Tricuspid valvePulmonary stenosisPulmonary regurgitation Primary pulmonary hypertension

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Acyanotic with a shunt

Shunt at atrial levelASDPAPVC

Shunt at ventricular levelVSD

Shunt at great artery levelPDAAP Window

Shunt at more than one level

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Acyanotic without a shunt Left sided malformations

Mitral stenosisMitral regurgitationAortic stenosisAortic regurgitationCoarctation of aorta

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Aortic Stenosis Age Symptoms Types

Valvular Supra valvular Sub valvularClinical PresentationManagement

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Coarctation of Aorta Age Types

Pre ductal Post ductal

Clinical presentation Associations Management

Age of intervention Surgery // Cath based intervention

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Acyanotic without a shunt Right sided malformation

Ebsteins anomaly of Tricuspid valvePulmonary stenosisPulmonary regurgitation Primary pulmonary hypertension

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Pulmonary stenosis Types Age Symptoms Clinical findings Management

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Ebsteins anomaly Age of presentation Clinical findings Management

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Acyanotic with a shunt




Shunt at more than one level

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VSD Types – based on location Size With or without PAH Associations Clinical features Management

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ASD Types Associations Clinical features D/D Management

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PDA Age Symptoms Signs D/D Management

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Cyanosis

Definition Central // PeripheralDifferential / Reverse differential

Causes Non Cardiac

RespiratoryVascular causes Hematological

Cardiac Congenital cyanotic heart disease

Eisenmenger’s Syndrome

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Cyanotic lesions

Tetralogy of Fallots Tricuspid atresia Transposition of Great arteries Truncus arteriosus Single ventricle Hypoplastic left heart syndrome Eisenmenger Syndrome

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Classification of Cong Cyanotic Heart diseases

Pulmonary stenosis, without VSDCritical PS, Ebsteins

Pulmonary Stenosis with Large VSDTOF

Increased Pulmonary flow with/ without PAHTGA ( Transposition of great arteries )

Decreased pulmonary flow with PAHEisenmenger Syndrome

Pulm venous congestion with PAH TAPVC, HLHS

Cyanosis without Pulm stenosis , Normal PA pressureSingle atrium, Pulm AV fistula

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Tetralogy of Fallots

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Transposition of Great Vessels

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Hypoplastic Left Heart Syndrome

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Clinical Characteristics of TOF

Prominent a wave in JVPNormal heart sizeMild parasternal impulse Systolic thrill – though uncommonSingle second sound ( P 2 absent )Ejection systolic murmur Diastolic period clearDecreased pulmonary flow on Chest X ray

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Congenital Cyanotic Heart diseases

Ejection Systolic Murmur / Pan systolic murmur Continuous murmur TOF with

PDABronchial collaterals Surgically created shunts ( BT shunt ) Peripheral PS

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DIFFERENTIAL DIAGNOSIS

Tetralogy of FallotTransposition of Great Arteries, VSD, PSTricuspid atresia, VSD, PSSingle ventricle, PSDouble outlet right ventricle, VSD, PSCorrected transposition of great arteries, VSD,PS AV Canal defects with PS Eisenmenger’s Syndrome

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Assessment of Severity

Cyanosis – More the cyanosis more severe the disease but mild cyanosis also to be taken seriouslyAge of onset – earlier the onset more severe the lesion Symptoms – more the symptoms more severe the disease

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Investigations

ECG X Ray Chest PA view Echocardiography Holter monitoring MRI Cardiac Cath

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ECG in CHD

Rate, Rhythm Look at P wave in Lead I PR interval QRS axis ( Left / Right ) RVH, LVH, Bivebtricular Hypertrophy Incomplete / Complete RBBB

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X Ray Chest

Cardiac Size C T Ratio Pulmonary Vascularity Classical images

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Echocardiography

2DEcho M mode Doppler

Pulsed wave, Continuous wave, Colour Tranesophageal ( TEE ) 3 D Echo

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Management of TOF

Age of presentation Severity of symptoms Anatomical considerations Palliative - Shunts Definitive

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Management of Complex CHD

Define anatomy by Echo, if required Cath/ MRI/ CT Angio

Decide whether Two Ventricle repair is possible or not

Glenn shunt Fontan repair

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Bidirectional Glenn

W What is the purpose of the Glenn?

I

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FontanFontan

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Reversal of shunt in following with development of Pulmonary arterial hypertension

Eisenmenger’s Syndrome




Shunt at more than one levelAV Canal defect

Patients with Cyanotic Heart diseases with Increased pulmonary blood flow – TGA, TAPVC

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Characteristics with Eisenmenger physiology

History of frequent chest infections

Age of onset of cyanosis?

No cardiomegaly or thrill

No parasternal heave

Constant ejection click of PAH

Palpable P 2

Diastolic murmur of PR or systolic murmur of TR

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Management of Eisenmenger’s Syndrome

Manage Polycythemia Drug management of PAH Heart Lung Transplantation

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Common Pediatric Cardiac Emergency Conditions Heart failure

Cyanotic Spell Duct dependent Circulation –

Pulmonary atresia , aortic atresia , TGA Cardiac Arrhythmias Post operative state

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CHF in Fetal Life

Supraventricular Tachyarrythmias AV Block AV Regurgitation Severe PR / TR EFE Severe anaemia Cardiomyopathy Myocarditis, Storage Diseases

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CHF on Day 1 of Life

Structural Heart Defects

Rhythm Abnormalities

AV fistulas

Heart muscle dysfuntion

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CHF in First week of Life

Structural Abnormalities Critical Aortic Stenosis Coarctation of Aorta Interrupted Aortic Arch Hypoplastic Left Heart Syndrome Critical Pulmonary Stenosis PDA in premature Babies

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CHF in First 2 months of LifeStructural Abnormalities Aortic level shunts Ventricular level shunts Left sided obstructive lesions Atrial level shunts Anomalous origin of Coronary Arteries

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Clinical Recognition Respiratory rate & Effort Heart Rate Feeding Difficulties Exercise intolerance Excessive sweating Weight gain Cardiomegaly Hepatomegaly

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Principles of Therapy

Removal of underlying cause - Surgical Correction

- Medical m/m of IE Removal of Precipitating cause - Intercurrent Infections

- Arryhthmias - Anemia

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DUCT DEPENDENT PULMONARY CIRCULATION DEFINITION Complete absence or severe restriction

of antegrade pulmonary blood flow resulting in severe hypoxemia with dependance on a patent arterial duct to maintain pulmonary perfusion compatible with life

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Duct Dependant Pulmonary Circulation ETIOLOGYA) Anatomical restriction/discontinuity of

ventricle and pulmonary arteryB) Admixture lesions- TGAC) Functional pulmonary atresia

Severe Ebsteins anomaly Hypoplastic right ventricle without PS

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DUCT DEPENDANT PULMONARY CIRCULATION

Clinical Presentation 90% have progressive hypoxemia,

cyanosis,acidosis 1-7 days after birth 5% present in

infancy/childhood,adolescent Rarely heart failure ( PS with TR, PA

intact septum)

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Clinical Presentation Blood Gas

Low Po2, Normal PCO2,Metabolic acidosis Hyperoxia test- Po2 < 150Torr

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DIAGNOSIS Echocardiography

Defines anatomy Duct size Pulmonary artery anatomy

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MANAGEMENT Early recognition- Key

Structural diagnosis- Less crucial

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MANAGEMENT Secure good I/V and I/A line

Correction of acidosis

Volume -colloid(5% Albumin 5-10ml/Kg)

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MANAGEMENT Prostaglandin- Life saving Oxygen(?) Balloon Dilation of duct Stenting of Duct Surgery

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MANAGEMENT Prostaglandin

Before transfer-0.01mcg/kg/min I/V Tertiary centre- 0.1mcg/kg/min scale to

0.05-0.01mcg/kg/min I/V Oral- 12-65 mcg/kg at 4hrly interval

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MANAGEMENT Prostaglandin- predictors of

response Widely open duct- Nil Closed duct-Nil Constricted duct- Best response

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DUCT DEPENDANT PULMONARY CIRCULATIONProstaglandin - side

effects Apnea Hypotension Fever Edema

Periostiitis (20%) Subcutaneous fat

necrosis Fragile ductus Aneurysm, rupture

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VENTILATION Transport to tertiary centre

Apnea of prostaglandin( premature, LBW)

Stabilisation of the sick child

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TRANSPOSITION COMPLEX

TGA intact septum/Small VSDDiagnosis- Echocardiography 100% detection rate ASD size- need for septostomy PDA size-need for prostaglandin

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TRANSPOSITION COMPLEX

TGA intact septum/Small VSDManagementa) Stable, no acidosis - BAS Plan Sxb) Acidosis,severe hypoxemia- PGE

BASc) Transfer- PGE (?Ventilation) BAS

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Surgical management

Arterial Switch Operation Venous Switch Operation

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CARDIAC ARRHYTMIAS

Complete Heart Block

Supraventricular Tachycardia

Ventricular Tachycardia - Rare

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CARDIAC ARRHYTMIAS

COMMONEST ARRHYTHMIA - SUPRAVENTRICULAR Well tolerated in majority In neonates and infants difficult to

differentiate from physiological response- fever, stress

Prolonged unrecognized SVT- DCM

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Clinical Presentation

Irregular Heart beat Unexplained cardiac failure Underlying structural heart disease Syncopal attacks Palpitations ,Chest discomfort Haemodynamic Instability Family H/O sudden cardiac events

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Long term treatment

Long term treatment till cath ablation is safe or LV dysfunction

Digoxin/B blockers Verapamil Amiodarone/Sotalol Flecanide

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Symptomatic second or third degree AV block

SA node Dysfunction , symptoms correlating with ↓ HR

Persistent ( > 7days) post op second or third degree AV block

contd….

Indications of Permanent Pacemaker Implantation

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Indications of Permanent Pacemaker Implantation

Congenital AV Block with a. Wide QRS escape b. HR < 50-55 bpm in infancy

without ass structural CHD c. HR < 70 bpm with ass structural

CHD

Therapeutic Cardiac Catherisation in Pediatrics

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GENERAL PRECAUTIONS Maintenance of ABC Maintenance of temperature Heparinization-100 units/kg- repeat if

reqd Arterial/ Venous approach Prostaglandins Oxygen

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INTERVENTIONS

Creation of a defect as palliation Balloon dilatation of stenotic valves Coil embolization / Balloon angioplasty Stenting of PDA Device closures

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Balloon Atrial Septostomy

Indications

Procedure

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BALLOON ATRIAL SEPTOSTOMY

ECHO GUIDEECHO GUIDE

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SUCCESSFUL SEPTOSTOMY Cinical improvement Equalization of atrial pressures Change in arterial O2 saturation Angiogram before and after septostomy Increase in arterial pressure Decrease in pulmonary arterial pressure Balloon calibration of defect Echo visualization of defect

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BALLOON DILATATION OF STENOTIC VALVES

Balloon dialtation of

Aortic valve

Pulmonary valve

Coarctation of aorta

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BALLOON DILATATION OF THE AORTIC VALVE Palliative procedure

Procedural success is almost 100%

Mortality may be high if associated conditions

Endocardial fibroelastosis LV hypoplasia Others

Risks- aortic regurgitation

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BALLOON DILATATION OF AORTIC VALVE

Indications

Procedure

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AORTIC VALVULOPLASTY

PRE DILATATIONPRE DILATATION BALLOON ACROSS BALLOON ACROSS THE AORTIC VALVETHE AORTIC VALVE

POST DILATATIONPOST DILATATIONNO ARNO AR

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BALLOON DILATATION OF PULMONARY VALVENeonatal critical Pulmonary stenosis Majority present during first week Duct dependant (require PGE1) RV morphology- well developed or

poorly developed Echo assessment Immediate success rate > 80%

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Tx PULMONARY VALVULOPLASTY

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Pulmonary valvotomy and VSD closurePulmonary valvotomy and VSD closure

Effective balloon diameter should be 1.2 to 1.4 times Effective balloon diameter should be 1.2 to 1.4 times the measured Pulmonary Valve Annulusthe measured Pulmonary Valve Annulus

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BALLOON DILATATION OF COARCTATION Controversial role in neonates Indications

Symptomatic newborn CCF Failure to thrive Upper extremity hypertension Severe LV dysplasia Severe PAH

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COIL EMBOLIZATION

Indications Large AVM Aortopulmonary collaterals Systemic venous anomalies PDA (if large, unrestrictive- surgery ideal)

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STENTING OF PDA

Ductal dependent pulmonary blood flow Difficult procedure Limited experience Intimal proliferation is universal thus

need for reintervention

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Device Closures

PDA ASD MUSCULAR VSD PERI MEMBRANOUS VSD IN OTHER PLACES e.g

Coronary AV fistulaPeri valvular leaks

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Closure of PDA

Device closure – for almost any duct Contra indications

Large PDA in a neonate

Associated reversal of shuntAssociated heart disease requiring

surgery PROCEDURE

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Coil closure of PDA

Coil closure for small ducts Coil made up of stainless steel wire with

Dacron strands – promote thrombosis Cheap

PROCEDURE

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DETACHABLE COIL

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Device closure of ASD

Amplatzer septal occluder device is most widely used

Only used for Secundum ASD Continuous TEE monitoring Adequate rim is required otherwise any size

of ASD can be closed

PROCEDURE

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(ASD)(ASD)

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Approach

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The Device

The device is made up of a NITINOL (Nickel-Titanium Naval Ordanance Laboratory).Dacron fibre - within the device

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Device closure of VSD

Amplatzer device for Muscular VSD Amplatzer device for Peri membranous

VSD – defect >5 mm away from aortic valve

PROCEDURE

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Pediatric Cardiology: Summary•1

•11938-Essentially no Rx for CHD–PPioneers-pathologists, Pediatricians, Cardiologists, Surgeons, Imaging experts, Intensivists, Interventionalists•22008-Rx for virtually all CHD, BUT mort./morb.:–vVentricular function–aArrhythmia– Valves/conduits–pPulm hypertension•N

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FUTURE OF INTERVENTIONAL CARDIOLOGY

1. Percutaneous pulmonary valve replacement2. Percutaneous aortic valve replacement3. VSD closure devices4. Percutaneous banding devices5. RF perforation6. Drug eluting stents7. Fetal intervention- therapy for aortic

stenosis

THANK YOU

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Healthcare

Cong heart diseasea