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Sanjay Gandhi Postgraduate Institute of Medical Sciences. Lucknow. Department of Cardiovascular & Thoracic Surgery and Telemedicine network at SGPGIMS. Welcomes participants in this presentation. "Birth defect of Heart, its Presentation and Treatment". Nirmal Gupta Head

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Sanjay Gandhi Postgraduate Institute of Medical Sciences

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Sanjay Gandhi Postgraduate Institute of Medical Sciences

Lucknow


Department of Cardiovascular & Thoracic SurgeryandTelemedicine network at SGPGIMS

Welcomes participants in this presentation


"Birth defect of Heart, its Presentation and Treatment"

Nirmal Gupta

Head

Department of Cardiovascular and Thoracic Surgery

SGPGIMS, Lucknow. U.P.


Magnitude: Birth defects of Heart in India

  • Every year 2 lakh children are born with congenital heart defects

  • At least 60,000 of these need treatment in the 1st year of life

  • Only 5000 get treatment because of lack of awareness amongst public in general and GP’s: delayed diagnosis

  • Poor socio-economic status of families: delayed treatment


Current facilities

  • Requires highly trained and dedicated team of diagnostic facilities, Surgeons and Nurses

  • Poor availability of facilities even in best hospitals

  • Not a financially viable option for private setups

  • Lack of trained manpower in the country (only 5 dedicated units other than SGPGIMS)


SYMPTOMS

"Birth defects of Heart, its Presentation and Treatment"


  • Neonatal History

  • Cyanosis

  • Failure to thrive

  • Exercise intolerance

  • Shortness of breath

  • Syncope

  • Palpitation

  • Chest pain


NEONATAL HISTORY

  • Cyanosis, shortness of breath.

  • Did the child need to stay in the hospital after maternal discharge?


  • Neonatal history

  • Cyanosis

  • Failure to thrive

  • Exercise intolerance

  • Shortness of breath

  • Syncope

  • Palpitation

  • Chest pain


CYANOSIS

  • > 5g/dl of deoxygenated Hb

  • False positive........... polycythemia

  • False negative...........anemia

  • Pathophysiology leading to cyanosis:

    • Obstruction of systemic venous blood flow to the lungs

    • Shunting of deoxygenated blood to left heart

    • Desaturation of systemic arterial blood


  • Neonatal history

  • Cyanosis

  • Failure to thrive

  • Exercise intolerance

  • Shortness of breath

  • Syncope

  • Palpitation

  • Chest pain


FAILURE TO THRIVE

  • Poor cardiac output and increased myocardial energy consumption coupled with poor feeding due to S.O.B.


  • Neonatal history

  • Cyanosis

  • Failure to thrive

  • Shortness of breath

  • Exercise intolerance

  • Syncope

  • Palpitation

  • Chest pain


EXERCISE INTOLERANCE

  • Baby................ poor ability to suck and feed

  • Child.................sedentary

  • Pathophysiology leading to exercise intolerance:

    • Poor cardiac output.

    • Increased energy consumption by an overworked heart.


  • Neonatal history

  • Cyanosis

  • Failure to thrive

  • Exercise intolerance

  • Shortness of breath

  • Syncope

  • Palpitation

  • Chest pain


SHORTNESS OF BREATH

  • Some children may be short of breath without appearing in distress "Happily tachypnoec"

  • Pathophysiology of S.O.B.:

    • Increase pulmonary blood flow

    • Interstitial edema

    • Decreased oxygen diffusion

    • Hypoxemia


  • Neonatal history

  • Cyanosis

  • Failure to thrive

  • Shortness of breath

  • Exercise intolerance

  • Syncope

  • Palpitation

  • Chest pain


SYNCOPE

  • Pathophysiology:

    • Inability to increase cardiac output suddenly due to restricted left ventricular outflow, e.g. severe aortic stenosis, IHSS.

    • Abnormal vasomotor tone resulting in vasodilatation when vasoconstriction is needed to maintain adequate blood pressure.


  • Neonatal history

  • Cyanosis

  • Failure to thrive

  • Exercise intolerance

  • Shortness of breath

  • Syncope

  • Palpitation

  • Chest pain


PALPITATION

  • Pathophysiology:

    • Irregular rhythm

    • Tachycardia

    • Awareness of normal rate and rhythm.


  • Neonatal history

  • Cyanosis

  • Failure to thrive

  • Exercise intolerance

  • Shortness of breath

  • Syncope

  • Palpitation

  • Chest pain


Rarely cardiac in origin.

Look for extra cardiac causes:

Skin,

Musculoskeletal,

Costochondral joints,

Pleural membranes,

Pericardium,

Referred pain

CHEST PAIN


SIGNS

"Birth defects of Heart, its Presentation and Treatment"


SIGNS

  • Inspection

  • Palpation

  • Auscultation


INSPECTION

  • Does the child appear ill?

  • Decreased tissue oxygenation due to poor cardiac output or severe cyanosis

  • Respiratory distress due to pulmonary edema or hypoxemia.

  • Cyanosis

  • Edema

  • Distended neck veins due to increased right heart pressure leading to systemic venous congestion

  • Clubbing of digits

  • Chronic peripheral tissue hypoxemia


SIGNS

  • Inspection

  • Palpation

  • Auscultation


PALPATION

  • Peripheral perfusion, normal 1-2 seconds.

  • Reflection of cardiac output.

    • FA=BA,

    • Normal = full

    • Diastolic runoff = bounding

    • Poor stroke volume = thready


PALPATION ( Contd.)

  • Precordium

  • Increased cardiac output, ventricular hypertrophy = hyperactive

  • Highly turbulent blood flow = thrill

  • Indicators of ventricular hypertrophy or atrophy = RV, LV impulses

  • Aortic stenosis, turbulent blood flow in ascending aorta = Suprasternal notch: thrill?

  • Hepatomegaly, check below right and left costal margins.


SIGNS

  • Inspection

  • Palpation

  • Auscultation


AUSCULTATION

  • LUNGS

    • Pulmonary edema = rales, crackles

  • HEART

    • First heart sound (S1):

      • Closure of atrio-ventricular valves.

    • Second heart sound (S2):

      • A2: closure of aortic valve

      • P2: closure of pulmonary valve

      • Single S2 = absent pulmonary or aortic component or delayed closure of A2 superimposing P2

      • inaudible P2 in TGA

    • Does the splitting of S2 vary with respiration?

      • Added sounds:

    • Gallop rhythm: S3, S4


AUSCULTATION (Contd.)

  • Murmurs

    • Grade: 1-6, one being the softest and six being the loudest.

    • By definition grade four murmur is associated with a palpable thrill.

  • Systolic murmur:

    • Holosystolic:

      Shunting of blood between two structures , the pressure in one structure is higher than the other throughout systole

      • Harsh: VSD

      • Soft: Atrio-ventricular valve regurgitation 

    • Ejection:

      Increase in blood flow turbulence as systole progresses due to an increasing amount of blood flow through a restricted orifice

      • Aortic stenosis

      • Pulmonary stenosis

      • Small VSD


AUSCULTATION (Contd.)

  • Mid-systolic:

    Increase volume of blood flowing through normal valves

    • ASD

    • Anemia

  • Diastolic murmur:

    Early:

    • Regurgitant blood flow from aorta or pulmonary artery into the ventricles

    • Aortic insufficiency

    • Pulmonary insufficiency

      Late:

    • Austin Flint murmur

    • Aortic regurgitation blood flow causes vibration of left ventricular free wall

  •  Systolic and diastolic murmur:

    Pressure difference between two structures during systole and diastole.

    • PDA & Shunts and collaterals


Congenital Heart Diseases and their Treatment

"Birth defects of Heart, its presentation and treatment"


Normal heart and its ECHO


Atrial Septal Defect: ASD


Coarctation of aorta


Ventricular Septal Defect: VSD


Patent Ductus Arteriosus: PDA


Pulmonary Stenosis: PS


Aortic Stenosis: AS


Atrio-Ventricular Canal:A-V Canal Defect


Tetralogy of Fallot: TOF


Transposition of Great Arteries: TGA


Transposition of Great Arteries: D-TGA


Truncus arteriosus: Truncus


Tricuspid Atresia: TA


Pulmonary Atresia: PA


Total Anomalous Pulmonary Venous Drainage: TAPVD


Hypoplastic Left Heart Syndrome: HLHS


Ebstein’s Malformation: Ebstein’s


General guidelines:

  • At birth

    • Blueness at birth or immediately after

    • Murmur of the heart

    • Rapid breathing

    • Low blood pressure


General guidelines:

  • At 2 – 6 months

    • Difficulty in feeding- baby is unable to suck properly, sweats or starts rapid breathing while feeding

    • Blue nails and toes and fainting spells

    • Inadequate weight gain

    • Recurrent chest infections


General guidelines:

  • In first 3 years of life

    • Fainting spells

    • Abnormal heart beats

    • Child avoids rigorous activities

    • Unable to play with his mates


General guidelines for couples

  • Drugs to avoid during pregnancy

    • Strict “NO” to Isoretinoin, Thalidomide, Estrogens, Oral contraceptives, ACE inhibitors, Chloramphenicol, Chlorpropamide, Erythromycin, Tetracycline and Haloperidol.

    • Anti-cancer drugs and Phenytoin are harmful but benefits outweigh the side effects.

    • Epinephrine, Ephedrine, B-blockers and Promethazine do not pose any significant risk, though the research is inadequate


In the end…

“There are a million times more patients in India with congenital heart diseases than polio, but the government’s budget for the treatment of congenital heart diseases is miniscule in comparison”


….so the gap is of more than billions magnitude


Thanks for your attention friends

Can I have your questions, please.


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