cyanotic chd

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Welcome to Seminar

Welcome to SeminarDr. Maimuna SayeedDr. Md. Tariqul Islam

Case ScenarioHistory4 weeks old male Gets bluish after feeding or crying Previously well, full-term babyThe family history was negative

Case Scenario (Contd.)PhysicalVigorous male, growing appropriatelyHR= 135, RR= 30, normal BP, no feverLungs clear to auscultation b/l, no wheezes, rhonchi, rales Purplish lips, hands and feetGrade III/VI systolic murmur loudest at lower left sternal borderLiver was 1.5cm below right costal margin and a normal spleenPeripheral pulses equal in upper/lower extremities, cap refill time < 3sec

Case Scenario (Contd.)WorkupPaO2 of 38mm Hg and a hyperoxia test showed increase to 48mm HgElectrocardiogram showed RVH

Case Scenario (Contd.)Chest X-ray

What could be the diagnosis

Congenital Cyanotic Heart Disease(Tetralogy of Fallot)

Congenital Cyanotic Heart Disease

OverviewCyanosis in newbornApproach to Cyanotic CHD

Cyanosis in NewbornIt is the bluish colouration of skin and mucous membrane due to increased deoxygenated haemoglobin in blood.Cyanosis becomes visible when there is >3-5g/dl of deoxygenated haemoglobin.- T. L . Gomella, 2013

Why Cyanosis Develops?The degree of cyanosis depends on both oxygen saturation and haemoglobin concentration.Cyanosis can be a sign of severe cardiac, respiratory or neurologic compromise.Cyanosis can also be caused by a reduced blood oxygen carrying capacity secondary to an abnormal form of hemoglobin such as methemoglobinemia.

Causes of Cyanosis in NewbornRespiratory Diseases:Parenchymal:Transient Tachypnea of Newborn (TTN)Hyaline membrane Disease (HMD)Aspiration - Meconium, blood, mucus or milkPneumoniaPulmonaryHaemorrhageEdema

Causes of Cyanosis in New-born (Contd.)Non-Parenchymal:Airway obstructionmucous plugchoanal atresiaExternal compression of the lungsany air leakspleural effusionCongenital defectscongenital diaphragmatic herniahypoplastic lungadenomatoid malformationdiaphragm abnormality

Causes of Cyanosis in Newborn (Contd.)Cardiac causes:Transposition of Great Arteries (TGA)Tetralogy of Fallot (TOF)Total Anomalous Pulmonary Venous Return (TAPVR)Truncus arteriosus Tricuspid atresiaEbstiens anomalySingle ventricleHypoplastic Left HeartDouble outlet right ventriclePersistent Pul HTN of Newborn (PPHN)

Five Ts

Causes of Cyanosis in New-born (Contd.)CNS cause:Hypoxic Ischemic EncephalopathyCongenital HydrocephalusSeizurePeriventricular-intraventricular hemorrhageMiscellaneous:MethemoglobinemiaPolycythemiaHypothermiaHypoglycemiaSepsisMetabolic acidosis

Making the diagnosisImmediate Questions: Does the infant have respiratory distress? Does the infant have a murmur? Was the infant cyanotic at birth? Is the cyanosis continuous, intermittent, cyclical, sudden in onset, or occurring only with feeding or crying?

Making the diagnosis (Contd.)Has the baby had the recommended pulse oximetry screening for CHD?Is there differential cyanosis?What is the prenatal & delivery history?

DiagnosisAfter through history and physical examination, investigations according to suspicion.Treatment according to cause.

Approach For Congenital Heart Disease In Newborn

Epidemiology of Congenital Heart DiseasesStructural Congenital Heart Disease is 6-8 per 1000 live birthsMajor cause of death during 1st year of life after prematurity (3/1000 live births)- John P. Cloherty, Manual of Neonatal Care: 7th EditionCongenital heart defects account for 27% of infant deaths worldwide.- American Heart association Update, 2013

Estimated causes of neonatal mortality around the year 2010 in Bangladesh

Data source: Bangladesh-specific mortality estimates (Liu et al. 2012).

Congenital Heart Disease documentation By NNPD In Bangladesh, June 2014 July, 2015

Etiology of CHDFactors associated with increased incidence:High risk of CHD in children:Prenatal factors:Genetic factors: Maternal rubellaRadiationAlcoholismAge >40 yrsInsulin dependent diabetesFetal intra uterine cardiac viral diseaseA sibling with a heart defectA parent with CHDChromosomal aberration e.g. Downs syndromeBorn with other congenital anomalies

Common Presentations Of CHDCyanosisCHFAsymptomatic heart murmurArrhythmia

Relevant Clues From History For CHDFamily History:The presence of a congenital heart lesion in a first-degree relative can have considerable diagnostic relevance. The recurrence rate in a first-degree relative is 1% to 4%. If there are two affected first-degree relatives, the recurrence rate is 3% to 12%.E.g. ventricular septal defect, patent ductus arteriosus, atrial septal defect, and tetralogy of Fallot- Arthur J Mos. 1992

HistoryMaternal & Perinatal History-Maternal DM (Ventricular septal hypertrophy) Maternal Rubella (PDA)Maternal exposure to alcohol or drugs

Physical ExaminationDysmorphic face (Trisomies 13, 18, 21)Colour- Pallor or cyanosisSigns of respiratory distress like tachypnea, chest indrawingPoor perfusionsPulse volume- bounding (PDA) or Diminished pulses in the lower extremities (COA)Blood pressure is 10 mmHg in arms than legs in COA

Physical Examination (Contd.)Abnormal heart rate (may be high or low than normal) Abnormal precordialactivity (Dextrocardia, cardiac enlargement, ventricular impulse)Abnormal S2splittingA single S2 occurs-Aortic atresiaPulmonary atresia (PA)Truncus arteriosus (TAC) Severe pulmonary stenosis (PS)

Physical Examination (Contd.)Abnormal extra heart sounds (gallop, pericardial friction rub)Pathologic murmurs (should be distinguished from innocent murmur)Hepatomegaly Extracardiac abnormalities

Diagnostic ToolsChest X-rayElectrocardiogramEchocardiogramArterial Blood GasHyperoxia testPulse oxymetry

Cyanotic heart disease Pulmonary Flow Pulmonary FlowTGATOFSingle VentriclePulmonary AtresiaTruncus ArteriosusTricuspid AtresiaTAPVR w/o ObstructionTAPVR With ObstructionSingle VentricleDouble Outlet Right VentricleHypoplastic Left Heart SyndromeEbstein Anomaly (of Tricuspid Valve)

Transposition of the Great Arteries

Complete Transposition of the Great Arteries5% of all CHD Male: Female = 3:1Most common cyanotic condition that requires hospitalization in the first two weeks of lifeAorta arises from the right ventriclePulmonary artery arises from the left ventricleDefect to permit mixing of two circulations- ASD, VSD, PDA.VSD is present in 50% of cases, necessary for survival

Clinical SymptomsNo mixing lesion and restrictive PFOProfound hypoxiaRapid deterioration Death in first hours of lifeAbsent respiratory symptoms or limited to tachypnea Single second heart sound, no murmurs

Clinical Symptoms (Contd.)Mixing lesion present (VSD or large PDA)Large vigorous infant CyanoticLittle to no respiratory distressMost likely to develop CHF in first 3-4 months of life

DiagnosisCXREgg shaped cardiac silhouetteNarrow superior mediastinumECGNormal or RVHEchocardiographyTransposed ventricular & arterial connection

ManagementProstaglandin to establish patency of the ductus arteriosusTherapeutic balloon atrial septostomy (Rashkind Procedure) if surgery is not going to be performed immediatelyImproves mixing and pulmonary venous return at the atrial levelDefinitive Surgery:Arterial switch operation (Jatene) - usually within first 2 weeks Mustard operation

Tricuspid Atresia

Tricuspid Atresia (Contd.)Represents about 2% of structural heart lesionTricuspid valve fails to developHypoplasia of right heartVenous blood from right atrium depends on open ASD or PFO, VSD, PDA

Tricuspid Atresia (Contd.)Clinical FindingsCyanosis at birthSingle S2Systolic murmur along left lower sternal borderDiagnosisChest X-Ray: Oligemic lung fields

Tricuspid Atresia (Contd.)ECGLeft axis deviationReveals left ventricular hypertrophyEchocardiographyAbsence of tricuspid valve

Tricuspid Atresia: TxPGE1 administration necessary Rashkind Balloon atrial septostomyPulmonary artery bandingGlenn shuntSuperior and inferior vena cava are connected directly to the pulmonary arteries - Between 3-6 monthFontan procedureAnastomosis between Right atrium & pulmonary artery - Between 1.5 3 year of age

Truncus Arteriosus

Truncus Arteriosus (Contd.)Failure of primitive truncus arteriosus to divide into aorta and pulmonary ArteryIt constitute 1% of CHDVSD almost always present4 types: According to arise of Pulmonary Artery from common trunk

Truncus Arteriosus (Contd.)Clinical Findings:Minimal cyanosis at birthCongestive Heart failure develops in 2-3 weeks Bounding pulses, pulse pressure widenedLoud, single S2Systolic murmur heard at left sternal borderDiagnosis:ECG reveals biventricular hypertrophyEchocardiography: Demonstrates the large truncal artery overriding the VSD and the pattern of origin of the branch pulmonary arteries

Truncus Arteriosus (Contd.)CXR Cardiomegaly Increased pulmonary vascularity

Truncus Arteriosus: TxSurgical repair (Rastelli repair)At 2 to 3 months of ageClosing VSDSeparation of pulmonary arteries from truncus Placing conduit between right ventricle and pulmonary arteries

Tetralogy of Fallot

Tetralogy of Fallot (Contd.)Constitute 10% of CHD Only 20% of TOF are cyanotic at neonatal periodVariable Degree of Pulmonary stenosis and size of VSD determine present degree of Cyanosis

Tetralogy of Fallot (Contd.)Pathophysiology:Increased resistance by the pulmonary stenosis causes deoxygenated systemic venous return to be diverted from RV, through VSD to the overriding aorta and systemic circulation systemic hypoxemia and cyanosis

Tetralogy of Fallot (Contd.)Symptoms:Dyspnea on exertion or when cryingTet spells: irritability, cyanosis, hyperventilation and sometimes syncope or convulsions due to cer

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