PresenterDr Gurulingappa

ModeratorDr Ranjan RK

Teratology of Fallot.Transposition of Great Vessels.

In 1888 Fallot described congenital heart defect composed of 4 characteristics. Large VSD Right ventricular outflow obstruction Overriding Aorta Right ventricular hypertrophy.

This malformation results from an anterior displacement of the cono septum

Pink tet- is a patient with TOF a source for adequate pulmonary blood flow

Without treatment ,25% of infants TOF &PS die in first year of life, 40% will die by the age of 4 years,70% by 10 years and 95% by 40 years.

Complex shunt with resistance to right ventricualr outflow directing blood Right to Left across VSD and leading to hypoxia and cyanosis.

Treatment 100% Oxygen and hyperventilation Knee chest postion. Intraoperatively open chest, direct compression

of aorta Morphine sulphate can be given to sedate the

patients and diminishing hyper apnoic response

Adequate maintenance of intravascular volume.

Beta blockers to reduce the infundibular spasm and heart rate.

If the child continues to be severely hypoxemic, SVR can be elevated and maintained with an infusion of norepinephrine and phenylephrine.

Sodiumbicarbonate.

History and Physical Examination History of hypercyanotic spells▪ Frequency▪ Severity

Medication history Investigations▪ Hb▪ Blood Group▪ Haematocrit.▪ Echocardiography▪ Chest Xray▪ Cardiac catheterization Data▪ Coronary angiography

Palliative Balloon dilatation of pulmonic valve Systemic and Pulmonary arterial shunts

Definitive Patch closure of VSD Ventriculotomy with reconstructionof

right ventricle outflow tractNPO and antibiotics

Goals of Management Maintenance of SVR Minimize PVR Avoid Myocardial depression Maintain Preloading.

Standard Monitors ECG SPO2 Invasive Blood Pressure ETCO2 Temperature Urine Output CVP monitoring Trans thoracic lines can be placed by the

surgeons before separation form CBP. Two large bore IV lines to be secured.

Cardiopulmonary Bypass and Or Deep hypothermic circulatory arrest.

Right Ventricular FailureResidual or unrecognized VSDResidual or unrepairable right sided

obstruction.Heart block and ventricular

dysarrythmias

TGA is a congenital heart disease, the great arteries are transposed as they relate to the heart.

The aorta arises form right ventricle and pulmonary artery arises from the left ventricle.

TGA accounts for only 5-7% of all congenital heart disease and has an incidence of 0.2 cases pre 1000 live births. If uncorrected TGA has 30 % mortality rate in first week of life, 45% in first month and 90% in first year.

Aorta arises form right ventricle, pulmonary artery from left ventricle.

VSD nearly half of the cases.Variable degree of sub pulmonic

stenosis.

Parallel right and left circuits, pulmonary blood flows to left ventricle and back to pulmonary artery without reaching systemic circulation.

Some mixing of pulmonary venous return across ASD, VSD, PDA required for survival.

History and Physical Examination Gestational age and birth complications Evaluation of the airway Arterial and intravenous access

Investigations Complete blood count Electrolytes Platelet count ABG Calcium , RFT, Glucose ECG Chest X ray. Blood Grouping and cross matching. Echocardiography Cardiac catheterization data. Coronary angiography

Atrial Baffle repair “ Mustard or Senning”

Anatomic correction with division of great arteries, reattachment to correct ventricular outflow- reimplantation of coronary arteries “Jatene”

VSD closure with left ventricle outflow to aorta and right ventricle to pulmonary artery conduit.

In the absence of an ASD or VSD, measures need to be taken to maintain ductus arteriousos

PGE1 Ballon atrail septostomy

Maintain preload, cardiac output and heart rate

Avoid myocardial depressionMaintain or decrease PVRAvoid reductions in SVR

Standard Monitors ECG NIBP Pulse oxymetry ETCO2 Temperature Urine output Arterial Line Trans esophageal echocardiography Two large bore IV lines.

CPB and or Deep Hypothermic circulatory arrest.

Atrail Baffle Systemic or Pulmonary Venous return obstructed Residual Intra-arterial shunts Systemic ( Right) ventricualr Dysfunction Systemic (Tricuspid) valvular dysfunction Atrial dysarrythmias.

Anatomic correction Inadequate preparation of left ventricle with left ventricular

failure Inadequate coronary flow, with myocardial ischemia or

infraction Stenosis of either great vessel anastomosis Aortic regurgitation

Rastelli Preocedure Obstruction to left ventricle Conduit obstruction Heart Block