Juan M Bolivar

a. b.

c.
d.

0.21 0.40 0.50 1.0

0%
a.

0%
b.

0%
c.

0%
d.

10

a.
b. c. d.

TAPVC HLHS Pulmonary atresia/VSD Critical coarctation of the aorta

0%
a.

0%
b.

0%
c.

0%
d.

10

a.
b. c.

d.
e.

TGA TAPVC HLHS Tetrology of Fallot Pulmonary atresia/VSD

0%
a.

0%
b.

0%
c.

0%
d.

0%
e.

10

Sepsis CHD Myocardial Ischemia : Hypoxia, ALCAPA Acquired or inherited cardiomyopathy Arrhythmia

Severe metabolic acidosis Lactic acidemia High WBC with left shift High creatinine Abnormal LFTs Normal electrolytes

Sudden closure PDA Systemic hypoperfusion Cellular hypoxia Severe increase on LV afterload Acute MR

Left side obstruction CoAo, Hypoplastic arch HLHS

Previously healthy Decrease appetite, poor suction Irritability vs. hypoactivity Cold, hypoperfused Low UO Tachycardia, hypotension

Vagal maneuvers Adenosine Amiodarone (F/U LFTs, Thyroid and lung function) Digoxin If combine amiodarone and digoxin, dose of digoxin should be decrease 50%

Viral myocarditis Perinatal hypoxia ALCAPA Inborn errors of metabolism Pompe is hypertrophic cardiomyopathy

The incidence of congenital heart disease in children with Down syndrome is up to 50 percent AV canal 60% VSD, ASD, PDA 20% TOF, HLHS 20%

PDA, VSD, AVC, AP window Increase volume and pressure in PA Heart failure High risk PHT Treatment: Diuretics, Afterload reduction, contractility Avoid oxygen, NO

Residual VSD PHT Heart block JET

In 1 to 3 % of all surgical procedures Mostly VSD, AVC, ToF 28 to 100 % mortality if untreated Typically transient resolves in 43 to 92% Most resolved within 10 days Treatment Pacemaker

The most frequent cardiac abnormality in is pulmonary valve stenosis, present in approximately 50% of the patients, followed by hypertrophic cardiomyopathy occurring in 25%

Is caused by the spontaneous deletion of 2628 genes on chromosome #7 (7q11.23 ) Supravalvar aortic stenosis (SVAS) is a fixed form of congenital left ventricular outflow tract (LVOT) obstruction

Congenital heart disease (40% of individuals), particularly conotruncal malformations (tetralogy of Fallot, interrupted aortic arch, ventricular septal defect, and persistent truncus arteriosus) Hypocalcemia Hypoplastic thymus

ASD PFO PAPVR RV dilatation High Qp/Qs Rare PHT

Cath devices; cardioseal, helex, amplatzer Surgical: Sinus node dysfunction Post pericardiotomy syndrome LV dysfunction

Narrow complex tachycardia with atrioventricular dissociation No P waves Cannon waves Low CO, high lactates

Correct hypovolemia, anemia and electrolyte imbalance Hypothermia or at least correction of fever. Cooling blankets, ice bags (sedation and paralysis required) Reduction in catecholamine's Atrial pacing Meds: Digoxin, Amiodarone

Broad spectrum from pink TET to PA/VSD/MAPCAS Most common cyanotic congenital anomaly 7-10% of congenital heart lesions

Pulmonary Stenosis Right Ventricular Hypertrophy Ventricular Septal Defect Overriding of the Aorta Boot shape X Ray

Evaluate for residual VSD, RV dysfunction and RVOT obstruction Evaluate for arrhythmia ( JET, Ventricular ectopy, heart block) Always keep good preload RV dysfunction: High CVP, low CO, high lactate, cyanosis

The Melody valve is made from a cows jugular vein valve that is sewn into a small metal stent

The Edwards SAPIEN Transcatheter Heart Valve (THV) is a heart valve that is made of cow tissue attached to a stainless steel mesh frame with a polyester wrap.

Preop: Good mixing BAS, PgE POP ASO LV dysfunction LA line Careful with IV fluids push Myocardial ischemia Coronary kinking ST segments

1.4 % of all congenital cardiac Association with 22q Pressure and volume overload of both ventricles Coronary ischemia sec to low diastolic pressure Repair in neonatal period POP: PHT, RV dysfunction (cyanosis), arrhythmias

Supra (50%) Cardiac (20%) or infradiaphragmatic(20%) Infradiaphragmatic usually obstructed Obstructed = Surgical emergency Does not improve with medical management Severe unresponsive cyanosis Prostaglandin can worsen cyanosis POP PHT

Normal Qp/Qs is 1.0 Plan: keep Qp/Qs close to 1.0 First days of life: High PVR keeps Qp/Qs normal PVR drops in first 72 h increasing pulmonary blood flow Qp/Qs > 2.0

Room Air, or hypobaric air with nitrogen Sedation, paralysis Bleed CO2 for mild hypercarbia Afterload reduction with milrinone

Excesive Pulmonary Blood Flow (Heart failure) More in pre surgical patients Increase PBF at expenses of SBF Final result decrease perfusion, metabolic acidosis, shock

Saturation > 85%

Pulmonary congestion
Low CO Low mixed venous saturation Increase lactic acid Low UO, acidosis Tachycardia, hypotension

Low PVR, look for oxygen, hyperventilation Shunt size Aortic arch obstruction Increase CO

DISBALANCE IN SINGLE VENTRICLE PHYSIOLOGY Sat < 75%

Inadequate PBF (hypoxemia) Causes: 1.No good intracardiac mixing 2.Restrictive PDA or BT shunt 3.High PVR 4.Pulmonary vein flow obstruction 5.Decrease mixed venous saturation

Saturation < 75%

Lung hypoperfusion
Cyanosis Low systemic circulation

Pulmonary venous desaturation: pneumothorax, pleural effusion, pulmonar edema, pneumonia, infection Systemic venous desaturation: anemia, increase oxygen consumption, low CO from decrease function or AV valve regurgitation Decreased PBF: High PVR, pulmonary venous hypertension, restrictive ASD, restrictive shunt, RPA or LPA stenosis

Left or right sided ductal dependent lesions The single ventricle may be a morphological right or left ventricle, or indeterminate There may be two good-sized ventricles where the inflow and/or outflow tracts cannot be separated

Tricuspid atresia/Pulmonary atresia DORV with left or right sided dominance HLHS Unbalanced AVSD L or D-TGA w/ HV Ebsteins anomaly Double inlet LV AVC with TGA

Same for all ductal dependent lesions unless nor single ventricle physiology requirements Requirements: Complete mixing at atrial level Blood will go to either circulation systemic or pulmonary based on resistance

Relationship Systemic to Pulmonary blood flow Based on Fick Principle Formula Ao Sat Mixed venous sat / PV sat - PA sat PA sat = Ao sat PV sat 100% Mixed venous sat= Aortic sat oxygen consumption (25%)

Aortic and PA sats are the same Pulmonary vein saturations 100% Oxygen consumption is constant No fever, no sepsis, not increase in metabolic rate No lung pathology

Ph 7.40 PaO2 40 Pa CO2 40 Ht 40

Ao sat 75 % 75-50 / 100 75 = 25/25 = Qp/Qs of 1 Aortic sat 90% 90-65 / 100 90 = 25/10 = Qp/Qs of 2.5 to 1 Aortic sat of 65% 65 -25 / 100 -65 = 25 / 35 = Qp/Qs of 0.7 to 1

RM BTShunt Central shunt Watterson and Potts shunt Norwood Damus Kaye Stansels (DKS)

Sat Low Decreased pulmonary venous saturation Decreased systemic venous saturation Decreased pulmonary blood flow Sat High Too much PBF Sat Appropriate Well balanced Too much PBF with dysfunctional lungs and/or systemic venous desaturation

Sano operation: RV to PA conduit Hybrid: septectomy, PDA stent, bilateral PA banding They dont fill criteria fro SV physiology

Elevated SVC pressures Hypertension/Bradycardia Hypoxemia Effusions Arrhythmias (sinus node dysfunction)

Fast track extubation: minimize intrathoracic pressure Mild hypercarbia to increase CBF Pain management decrease metabolic rate Treat hypertension; Afterload reduction increases CO Oxygen decreases PVR (Sildenafil,NO)

Mechanical ventilation Low CO Arrhythmias Cyanosis Effusions Thrombosis

Fenestrated ? Fast track extubation Low CO: Preload, Elevated PVR, anatomic obstruction, pump failure Arrhythmias Cyanosis Effusions

a. b.

c.

d.

Lower the FiO2 Paralyze, sedate and control minute ventilation Begin milrinone infusion if the blood pressure permits Consult surgery for an emergent operation

0%
a.

0%
b.

0%
c.

0%
d.

10

a. b.

c.
d.

0.21 0.40 0.50 1.0

0%
a.

0%
b.

0%
c.

0%
d.

10

Q2-The most appropriate FiO2 to ventilate an unoperated baby with HLHS in shock and an SaO2 of 76% is:
0.21 100 100

0.4

100 100

0.5

100 100

100 100
First Slide Second Slide

a. b.

c.
d.

Cyanosis Shock SVT Hydrops

0%
a.

0%
b.

0%
c.

0%
d.

10

Small ductus with restrictive flow, Increased PVR secondary to parenchymal disease, Increased pulmonary venous pressure secondary to obstructed pulmonary venous drainage, Restrictive ASD

a.
b. c. d.

TAPVC HLHS Pulmonary atresia/VSD Critical coarctation of the aorta

0%
a.

0%
b.

0%
c.

0%
d.

10

a.
b. c.

d.
e.

TGA TAPVC HLHS Tetrology of Fallot Pulmonary atresia/VSD

0%
a.

0%
b.

0%
c.

0%
d.

0%
e.

10

Q5-In an infant with a cyanotic congenital heart disease, nitrogen may be used for preoperative stabilization of the baby with which one of the following diagnoses?
TGA 100 100 100 100 100 100 100 100 100 100
First Slide Second Slide

TAPVC

HLHS

Tetrology o...

Pulmonary a...

a.

b.

True False

0%
a.

0%
10
b.

a. b. c. d.

increase the FiO2 decrease the ventilator rate increase his vasodilators increase his inotropes
0%
a.

0%
b.

0%
c.

0%
d.

10

a. b. c. d.

increase pulmonary blood flow decrease pulmonary blood flow increase systemic oxygen delivery none of the above

0%
a.

0%
b.

0%
c.

0%
d.

10

a. b. c. d.

increase pulmonary blood flow decrease pulmonary blood flow increase systemic oxygen delivery none of the above
0%
a.

0%
b.

0%
c.

0%
d.

10

a. b. c. d.

e.

Patch augmentation of the aortic arch Systemic to pulmonary shunt Atrial septectomy Transfer of coronary arteries from aorta to pulmonary artery Anastomosis of pulmonary artery to aorta

0%
a.

0%
b.

0%
c.

0%
d.

0%
e.

10

a. b.

c.
d.

Hypertension Irritability Sinus node dysfunction JET

0%
a.

0%
b.

0%
c.

0%
d.

10

a. b. c.

d.
e.

Keeping the head elevated Increasing the FiO2 Aggressive hyperventilation (target pCO2 25) Gentile hyperventilation (target pCO2 35) Gentile hypoventilation (target pCO2 45)

0%
a.

0%
b.

0%
c.

0%
d.

0%
e.

10

a.
b.

c. d.

Residual VSD Restrictive bulboventricular foramen Branch PA stenosis Diminished diastolic ventricular compliance
0%
a.

0%
b.

0%
c.

0%
d.

10

a. b. c. d.

increase the milrinone Begin dopamine infusion Give volume infusion Do nothing, the data is acceptable
0%
a.

0%
b.

0%
c.

0%
d.

10

a.

b.

True False

0%
a.

0%
b.

10

a. b. c. d.

VSD repair Norwood operation Bidirectional Glenn operation Repair of TOF

0%
a.

0%
b.

0%
c.

0%
d.

10

a. b.

c.
d.

Amiodarone Lidocaine Procainamide Adenosine

0%
a.

0%
b.

0%
c.

0%
d.

10

a. b. c. d.

AV reciprocating tachycardia Ventricular tachycardia JET Sinus tachycardia

0%
a.

0%
b.

0%
c.

0%
d.

10

a. b. c. d. e.

Cooling Adenosine Amiodarone Procainamide and cooling Pacing

0%
a.

0%
b.

0%
c.

0%
d.

0%
e.

10

Narrow complex AV dissociation Ventricular rate faster than atrial rate Causes low cardiac output

Treatment:
Pacing Hypothermia Amiodarone Procainamide Sedation Reduce adrenergic drugs

a. b. c. d.

4 month old after bidirectional Glenn 7 day old after repair of truncus 2 month old after Mustard operation 2 year old after repair of sinus venosus ASD
0%
a.

0%
b.

0%
c.

0%
d.

10

a. b. c. d. e.

Norwood operation Bidirectional Glenn operation Fontan operation Arterial switch operation None of the above

0%
a.

0%
b.

0%
c.

0%
d.

0%
e.

10

a. b. c. d.

Norwood operation Bidirectional Glenn operation Fenestrated Fontan operation Arterial switch operation

0%
a.

0%
b.

0%
c.

0%
d.

10

a. b. c. d.

Who has a VSD closed at 8 mths Who has PFC Who has an endocardial cushion defect repaired Who has fixed mitral stenosis and pulmonary hypertension

0%
a.

0%
b.

0%
c.

0%
d.

10

a.

b.
c. d.

Arterial switch Norwood Damus-KayeStanzel Bidirectional Glenn

0%
a.

0%
b.

0%
c.

0%
d.

10

a. b. c. d.

Arterial switch operation Norwood operation Bidirectional Glenn operation Hemi-Fontan Operation

0%
a.

0%
b.

0%
c.

0%
d.

10

a. b. c.

d.

A 6 year old having a coarctation repair A newborn after repair of TAPVC A 6 month old after bidirectional Glenn repair A neonate after Norwood operation
0%
a.

0%
b.

0%
c.

0%
d.

10

a. b. c.

d.

8 mth old undergoing VSD repair 8 mth old undergoing bidirectional Glenn repair 8 mth old undergoing repair of truncus arteriosus 3 year undergoing Fontan operation with underlying diagnosis of HLHS

0%
a.

0%
b.

0%
c.

0%
d.

10

a. Small, kinked BT shunt b. Ventricular dysfunction c. Myocardial ischemia d. Residual arch obstruction e. AV valve regurgitation
0%
a.

0%
b.

0%
c.

0%
d.

0%
e.

10