RNC cardiac review

Elizabeth Rex, MS, NNP - BC NCC Cardiac Content

♥ Congestive Failure ♥ Transition to extrauterine life ♥ Hypertension ♥ PDA ♥ ♥ CV Assessment BP CVP ♥ EKG Monitoring ♥ Anomalies (Cyanotic / Acyanotic) Lines AV Canal ♥ Cyanosis Coarctation of aorta Central / Peripheral HLHS Cardiac / Pulmonary Pulmonary stenosis/atresia TOF ♥ Arrhythmias TGA TAPVR Fetal Circulation

3 fetal shunts Ductus venosus Foreman ovale Embryonic Development

 Cardiac septation : begins middle of the 4 th week and complete by end of 5 th week  Defects arising from problems in septation : VSD, ASD, endocardial cushion defect (AV canal), malformation of tricuspid and mitral valves

Great Vessel Development

 Happens simultaneously with septation  Defects that occur with great vessel development:  Truncus Arteriosus  TOF  Pulmunary and malformations  Transposition  DORV Cardiovascular Transition

♥ 10 min = PaO2 50 mm hg ♥ 1 hr = PaO2 62 mm hg ♥ 2 days PaO2 75 - 85 mm hg

24 hours after birth: ♥ Oxygen consumption triples ♥ Significant increase in ♥ Left must remodel and hypertrophy Respiratory Assessment

♥ Normal Rate: 30 - 60, easy effort

♥ Increased WOB: tachypnea, GFR, gasping

♥ S aturations: Pre and post Assessment

♥ Normal rate 120 - 160 (may range 80 - 200)

♥ Normal sinus rhythm

Underlying causes ♥ Vagal response ♥ Apnea ♥ Hypoxemia ♥ Asphyxia ♥ H ypotension ♥ Acidosis ♥ Digoxin toxicity ♥ Central line in right atrium

Evaluate for shock ♥ HR < 70 is usually pathologic ♥ D ifferentiate Sinus bradycardia - QRS complex follows each p wave ♥ Complete heart block Complete Heart Block

♥ Ventricular rate 45 - 89 beats/min

♥ P wave unrelated to QRS

♥ I ncreased incidence with maternal lupus erythematosus

♥ If hydroptic at birth, will be critically ill

♥ Abnormal tachycardia sustained HR>180 - Assess for shock, CHF - Evaluate resp status, , , BP

♥ Most common: sustained HR 180 - 220 Sympathetic stimulation Fever SVT Supraventricular Tachycardia

Sustained HR > 220 ♥ Usually well tolerated initially unless associated with:

Structural CHD

Hydrops SVT

- 15 lead EKG - Run while doing tx - Vagal maneuvers - Stimulate a gag - Suction nasopharynx - Ice to nose and forehead - Adenosine - Initial dose 100 mcg/kg - Rapid IV push over 1 - 2 seconds followed by flush - No response in 2 minutes increase dose 50 - 100 mcg/kg - Cardiover sion - 0.5 joules/kg Heart

♥ First heart sound – S1

 Closure of mitral and tricuspid valves  End of atrial S1

 Accentuated, may mean:  Increased CO  PDA, VSD, TAPVR, TOF  AVM  Anemia  Fever  Diminished, may mean:  CHF  Myocarditis Heart Auscultation

Second heart sound – S2  Closure of aortic and pulmonic valves  End of ventricular systole  is a normal finding and just reflects the aortic valve closing before the pulmonic valve. S2

 First 48 hours  Normal to hear single S2 in first two days of life because of increased PVR. If you hear a split S2 at birth, could indicate abnormalities of P or A valves or alterations in PVR and SVR. • After 48 hours • S2 split elongated • ASD, TAPVR, TOF, Ebstein’s anomaly • Absent split • , PPHN, TGA, TA S3 & S4

 S3: O nly heard in left to right shunts and mitral valve insufficiency  S4: Should not be heard in newborn. If so, indicates decreased ventricular compliance

 Ejection clicks: Abnormal after 24 HOL and heard after S1. Associated with dilation of great vessels or malformation of PV and AV

Sound caused by turbulent blood flow

♥ Blood forced through narrowed areas

♥ Regurgitation through incompetent or abnormal valves

♥ Increased flow across normal structures Heart Auscultation

Murmurs  Location  Transmission  Intensity  Timing  Quality  Grading I - VI Heart Murmur Intensity

Grade I - barely audible Grade II - soft but audible Grade III - moderately loud, no thrill Grade IV - loud, assoc. with thrill Grade V - audible with stethoscope barely touching chest Grade VI - audible with stethoscope not touching chest Heart Murmur Timing

♥ Systolic  Heard between S1 and S2 of same beat S1 (murmur) S2 S1 (murmur) S2

♥ Diastolic  Heard between S2 and S1 of next beat S1, S2 (murmur) S1, S2 (murmur)

♥ Continuous  Starts in systole and extends into Normal/Innocent Murmurs Up to 50% of neonates can have a murmur in the first 48 hours of life

♥ Continuous systolic/Crescendo systolic murmur ( L R flow through PDA)

♥ Early soft midsystolic ejection murmur aka Peripheral pulmonic stenosis (PPS)  Grade I - II/VI (no thrill) upper left sternal border, radiates to axilla and back

♥ Systolic ejection murmur (turbulence of blood flow across ) Grade I - II/VI may be heard 1 st week of life as PVR decreases and PDA closes Pathologic Heart Murmur ♥ > Grade 3 murmur within hours of birth

SYSTOLIC • Pan systolic murmur • Mitral or tricuspid regurgitation • VSD DIASTOLIC • Aortic or pulmonic valve regurgitation

Continuous murmur PDA, AVM, Pathologic Heart Murmur

♥ Central cyanosis ♥ Respiratory distress ♥ Abnormal heart silhouette ♥ ↑ or ↓ pulmonary vascularity on CXR ♥ Gallop  CHF

Assessment - Obeservation

♥ Skin: color, temperature, diaphoresis, edema

♥ Precordium: quiet, visible, heave, thrill PMI - LLSB 5 th intercostal space PMI shifted to the right: Tension pneumothorax Diaphragmatic hernia

PMI shifted to the left Tension right pneumothorax Assessment - Palpation

♥ Pulses: compare upper to lower extremities and side to side (if not equal, could mean LOTO)  R Brachial and femoral equal in strength (R brachial = R subclavian = pre - ductal)  Pedal pulses palpable  Weak: LOTO, myocardial failure or shock  Bounding (=“aortic runoff”): PDA, , systemic to pulmonary shunt

♥ CFT: press for 5 seconds, release < 3 seconds normal Assessment Auscultation

Bruits ♥ Liver ♥ Anterior fontanelle ♥ M ay indicate AVM arteriovenous malformation Cyanosis - Peripheral

Results from sluggish movement of blood to the extremities and increased tissue oxygen extraction

♥ Acrocyanosis  Bluish discoloration hands and feet  No mucous membrane involvement  Often resolves by 48 hours of age  Rule out hypothermia Cyanosis - Peripheral

♥ Circumoral cyanosis  Bluish discoloration around the mouth  Often associated with feeding  R/O central cyanosis Cyanosis - Central

= deoxygenated blood leaving the

♥ Bluish discoloration of tongue and mucous membranes

♥ Caused by desaturation of arterial blood. Hemoglobin carrying no O2 appears purple.

♥ At least 5g of desaturated hemoglobin/dl is necessary before you can observe cyanosis.

♥ Also influenced by presence of anemia or polycythemia

♥ Indicates cardiac or respiratory dysfunction Cyanosis - Pulmonary / Cardiac

Pulmonary Cardiac Cyanosis Yes Yes Respiratory Rate Increased Increased – often tachypneic no GFR Infant looks comfortable if no C HF Work of breathing Increased Easy effort unless CHF – then GFR Acid/Base Balance Increased PCO2 Decreased PCO2 with tachypnea Respiratory acidosis Metabolic acidosis Mixed resp/metabolic if pulmonar y disease CXR Asymmetric pattern of Increased or decreased infiltrates or other pulmonary vasculature pulmonary disease Heart silhouette normal abnormal Size/shape/location O2 Challenge test PO2> 150 PO2 < 150 for cyanotic CHD Blood pressure

Methods for measuring

Arterial:

♥ Umbilical

♥ PAL Radial

♥ PAL Posterior tibialis Discrepancies in upper and lower BPs

 Coarctation of the aorta  Any type of arch abnormality  PDA Hypertension

♥ Systolic or mean arterial BP > 95 th percentile for birth weight, gest age, and post - natal age

95 th percentile for systolic BP = 65 mmHg at 24 wks.

95 th percentile for BP = 90/60 mmHg at 40 wks post conception.

 Renal abnormalities most common cause Hypertension Treatment

♥ V aries with cause of hypertension

♥ T x etiology if possible

♥ A nti - hypertensive only if hypertension immediately life threatening CVP

♥ In most cases the trend in CVP is more helpful than absolute value

♥ CVP may be difficult to interpret because it is affected by several factors:  hypervolemia  myocardial failure  excessive ventilatory pressures  grunting respirations  tension pneumothorax  pleural effusion  UVC tip in portal system Systole & Diastole

Adult

Systole is contraction of the ventricles

Diastole is the relaxation and filling of the ventricles followed by a small atrial contraction Cardiac Cycle

average neonate’s cardiac cycle is ≈ 0.4 secs, based on a HR of 150

Supraventricular tachycardia HR 230 60 ÷ 230 = 0.26 secs HR 300 60 ÷ 300 = 0.2 secs C ardiac Output The volume of blood pumped by the left ventricle in 1 min 120 - 200 ml/kg/min

CO = x HR Cardiac Output

I nfluenced by changes in HR, pulmonary , and systemic vascular resistance to flow

A lso influenced by the amount of blood returning to the heart Stroke Volume R elatively fixed at 1.5 ml/kg

Factors that affect SV

♥

♥

♥ Contractility Preload

T he volume of blood in the ventricle before contraction (or at the end of diastole – aka end diastolic pressure)

C linically, a measure of pressure rather than volume

Dependent upon venous return to the heart

An ↑↓ in preload can significantly affect CO in the neonate’s non compliant heart Preload Changes

↓ ♥ ♥ Intrapartum blood loss ♥ Peripheral pooling secondary to bacterial sepsis

↑ ♥ Fluid overload ♥ Left to right shunt thru PDA, VSD, PFO Contractility

S peed of ventricular contraction (systole) - Intrinsic pumping ability

Neonate’s heart has a limited capacity to increase contractility

C annot be clinically measured Contractility

Increased by: exogenous catecholamines (Dopamine and Dobutamine )

Decreased by: Acidosis Hypoxia Hypocalcemia Hypoglycemia Hypercarbia Myocarditis R esistance to blood leaving the ventricles

D ependent on the systemic vascular resistance and pulmonary vascular resistance (increase in SVR or PVR = afterload)

A fter - load can be reduced by IV infusions of vasodilators

Cardiovascular function is modulated by the autonomic nervous system Baroreceptors

Baroreceptors and chemoreceptors in the aorta and carotid sinuses provide feedback to the autonomic nervous system The parasympathetic or sympathetic nervous systems are then stimulated Sympathetic stimulation through the ganglionic chain releases norepinephrine and epinephrine which act on the SA node, the AV node, the atria and the ventricles. Alpha - and beta - adrenergic receptor stimulation alpha - ↑ contractility and ↑ rate beta – vasodilatation, bronchodilation, and smooth muscle relaxation Congestive

♥ Myocardial dysfunction in which the heart is unable to pump enough blood to meet its needs, to dispose of venous return adequately, or a combination of the two

♥ M ay result from CHD or acquired heart diseases with volume or pressure overload or from myocardial insufficiency Potential Causes

♥ Structural cardiac defects ♥ Hypoglycemia ♥ Cardiomyopathy ♥ Hypocalcemia ♥ Cardiac arrhythmias ♥ Severe anemia ♥ AV malformations ♥ Polycythemia ♥ Multiple hemangiomas ♥ Fluid overload ♥ Asphyxia ♥ Renal Failure ♥ Bronchopulmonary dysplasia ♥ Adrenal insufficiency ♥ Sepsis ♥ CHF - fetal causes

♥ SVT Presentation: ♥ Severe bradycardia d/t Hydrops complete heart block ♥ Anemia Treatment: ♥ Ebstein’s anomaly Digoxin ♥ Myocarditis CHF - occurring the first day of life

♥ Asphyxia ♥ HLHS ♥ Hypoglycemia ♥ Severe tricuspid or pulmonary regurgitation ♥ Hypocalcemia ♥ Sepsis ♥ Systemic AVM CHF - occurring the first week of life

♥ PDA ♥ TGA ♥ Adrenal Insufficiency ♥ TAPVR ♥ Closure of PDA with ductal dependent lesion: Coarctation of the aorta Hypoplastic left heart syndrome Interrupted aortic arch CHF - occurring beyond the second week of life

♥ VSD most common reason

♥ Truncus arteriosus CHF – pulmonary presentation

♥ Poor weight gain *** ♥ P oor feeding of recent onset *** ♥ Feeding intolerance ♥ T achypnea, dyspnea that worsens during feeding *** ♥ Increased WOB: Grunting, flaring, and retracting Head bobbing ♥ Rales, rhonchi, wheezing *** ♥ Irritability, lethargy ♥ Pulmonary infiltrates on CXR Pulmonary venous engorgement (aka )

Cause is blood backing up in the pulmonary system Leakage of fluid into the pulmonary interstitium

• Interferes with gas exchange

• LV dysfunction &/or overload may increase LV end diastolic pressure CHF – CV presentation ♥ C old sweat on forehead - diaphoresis ♥ P uffy eyelids, dependent edema ♥ Pallor, mottling, cyanosis ♥ Increased precordial activity ♥ Peripheral pulses initially full but decrease in end stage, prolonged CFT ♥ T achycardia *** , , BP changes ♥ Decrease in urine output (<0.5 ml/kg/ hr ), increase in specific gravity ♥ Sudden w eight gain in end stages ♥ H epatomegaly *** ♥ C ardiomegaly on CXR *** Systemic venous engorgement

Blood backing up in the systemic system

Leakage of fluid into the periphery interstitium and liver

Hepatomegaly

RV dysfunction &/or overload my increase RV end diastolic pressure CHF – SNS compensatory mechanisms

↓ Systemic BP activates baroreceptors

↑ sympathetic stimulation

♥ ↑ HR

♥ ↑ cardiac contractility

♥ ↑ arterial BP CHF – SNS compensatory mechanisms Catecholamine release ↑ venous tone ↑ blood return to the heart

↓ circulation to skin, kidneys, extremities, & splanchnic bed

↓ Renal blood flow stimulates release of renin angiotensin, and aldosterone

which triggers retention of Na and fluid, resulting in increased circulating blood volume

↑ Blood volume puts additional work load on the heart CHF – mechanical compensatory mechanisms

Cardiac muscle thickens to ↑ myocardial pressure

Hypertrophy effective in early stages as muscle mass ↑ compliance ↓

↓ compliance requires ↑ filling pressure for CO CHF – mechanical compensatory mechanisms

Hypertrophied heart eventually becomes ischemic

Ventricular dilation occurs to accommodate volume

Initially the heart tries an increased force of contraction but soon fails CHF - management

♥ Prone position

♥ Decrease oxygen consumption  NTE  Minimize stimulation  Provide sedation  Assisted ventilation  Supplemental O2  Correct acidosis and electrolyte imbalances CHF - management

 Fluids and nutrition  Fluid restriction  limiting PO feeding  increased calorie feedings Medications for CHF

Diuretics: Furosemide (Lasix ) Hypochloremia and hypokalemia can lead to metabolic alkalosis. Can also cause hypocalcemia . Spironolactone ( Aldactone ) K+ sparing, but can cause hyper kalemia Chlorothiazide ( Diuril ) K+ and Ca sparing; not as powerful as a diuretic Medications for CHF

Digoxin:

 positive inotrop ic ↑ cardiac contractility  negative ↓ HR

 Observe for bradycardia , arrythmias , hypokalemia

Inotropes:  Dobutamine , dopamine Shock

♥ A n acute state in which circulatory function is inadequate to supply sufficient amounts of O2 and nutrients to meet metabolic demands

♥ I n most cases, cardiac output is low

♥ I n early shock, compensatory regional may temporarily maintain normal BP Hypotension

♥ Late sign - cardiac decompensation

♥ Treatment of shock based on more than BP: Evaluate history

Physical exam

Labs Shock

♥ As shock progresses, compensatory mechanisms fail and there is widespread cellular damage

♥ I nsufficient delivery of O2 results in anaerobic metabolism and lactic acidosis. Shock

♥ I f shock persists, irreversible injury to vital organs occurs, death ensues despite vigorous treatment that may temporarily return cardiovascular measurements to normal Shock Etiology

♥ Hypovolemia (Hypovolemic shock)

♥ A sphyxia ()

♥ C ardiogenic causes

♥ S epsis ()

♥ D rugs Hypovolemia

• Blood loss ( intrapartum or postnatal) • Inadequate placenta transfusion • Severe dehydration • Third spacing • Skin integrity losses ( gastroschisis ) • Pleural effusions ( hydrops ) Asphyxia

Antepartum

Intrapartum

Respiratory failure

Impaired O2 transport due to severe anemia or hemoglobinopathy Cardiogenic Cardiomyopathy

Dysrhythmias

Congenital malformation

Hypocalcemia

Severe hypoglycemia Sepsis

Especially early onset group B beta - hemolytic Streptococcal Drugs

1. Maximize cardiac output 1. e pinephrine 2. dopamine 3. d obutamine 4. Milrinone 5. Isoproterenol (increases HR and contractility) Shock Presentation Cardiovascular

 Systemic arterial hypotension

 Narrow pressure

 Central venous hypotension, although CVP may be elevated with cardiomyopathy

 Tachycardia Shock Presentation

Respiratory Other signs

 Tachypnea  Prolonged  Retractions capillary fill time  Grunting  Oliguria  Apnea  Hypothermia  Metabolic acidemia Cardiac Tamponade – a medical emergency

♥ T he hemodynamic result of fluid accumulation in the potential space surrounding the heart or .

♥ E xcessive fluid accumulation results in ↑ pericardial pressure, causing ↓ ventricular filling, ↓ cardiac output and hypotension.

♥ The rapidity of fluid accumulation influences the hemodynamic effect.

♥ Beck’s triad (jugular venous distension, hypotension, and muffled ).

Congenital Heart Defects

 85% multifactorial  10% chromosomal  2% genetic  2% maternal or environmental teratogens Chromosomal abnormalities

 You will often see cardiac defects with:  Trisomies (esp. 18 and 13)  Turner syndrome  Noonan’s  DiGeorge  22q11 Environmental Factors and Teratogens

 Thalidomide  Anticonvulsants (Phenytoin, Carbamazepine, Valporic Acid, Pentobarbital, Trimethadione )  Anticoagulants (Warfarin, Heparin)  Lithium  Alcohol  Amphetamine Maternal Disease and Viral Infections

 Diabetes: 5 x’s the risk of CHD (VSD, DORV, TGA, TA and coarctation )  Lupus (heart block)  Rubella and CMV (PDA, PS, VSD, ASD)  Obesity Congenital Heart Disease

Increased Pulmonary Flow: PDA - VSD – Ventricular septal defect AV Canal – Endocardial Cushion partial or complete 30% are infant’s with trisomy 21 ASD – Congenital Heart Disease

 Obstructive Lesions that cause Pulmonary Venous Congestion

 Coarctation of the aorta

 Aortic Stenosis Congenital Heart Disease

Ductal dependent lesions that decrease pulmonary blood flow:

TOF - with severe PS or PA PA - PS - Pulmonary Stenosis TA - Congenital Heart Disease

Mixed lesions: TGA - Transposition of the great

TAPVR - Total anomalous pulmonary venous return

HLHS - Hypoplastic left heart syndrome

TA - Truncus arteriosus Ductal Dependent Defects

♥ Need to have PDA open for systemic or pulmonary circulation

♥ PGE 1 continuous infusion to open and maintain ductal patency PDA

Shunts right to left flow in utero

Persistent PDA will shunt left to right as PVR decreases

PGE used to open PDA: If you need left to right flow for pulmonary perfusion If you need right to left flow for systemic perfusion Pathologic PDA

 Symptoms:  Increased pulmonary vasculature and cardiomegaly on CXR  Bounding peripheral pulses  Active precordium  Widened  Low diastolic BP Indomethacin

 Prostoglandin inhibitor  Complications  Decreased renal blood flow = oliguria  Increased incidence of GI bleed  Thrombocytopenia = increased chance of ICH Diagnostics: EKG cardiac depolarization is the result of an electrical discharge across the myocardial cell cardiac depolarization is measured by the EKG reflects abnormal hemodynamic burdens placed on the heart right ventricular prominence normal after birth major tool to evaluate arrhythmias and the impact of electrical imbalances Diagnostics: EKG

 Tall, peaked P waves are seen in RIGHT sided heart failure Diagnostics: EKG

Wide, notched P waves seen in LEFT sided heart failure Diagnostics: Blood Gas

Hyperoxygen Test 1. ABG @ RA 2. 100% FiO2 x 10 mins 3. @ 10 mins , draw ABG 4. If significant bump in PaO2, likely respiratory. If not, Diagnostics: X - ray

 Cardiomegaly: Cardiothoracic ratio > 65%

 “Egg on a string” = TGA Diagnostics: X - ray

 TOF = “boot - shaped” heart Diagnostics: X - ray

 TAPVR = “snowman” appearance Echocardiogram

The Gold Standard

P rovides rapid, non - invasive, and painless evaluation of the  anatomy and flow by the use of ultrasonic waves Cardiac Catheterization

♥ I nvasive procedure to obtain data for a definitive diagnosis, intervene, or to prepare for surgery

 Diagnostic Evaluate Selective angiography

 Interventional Balloon atrial septostomy (Rashkind) Balloon valvuloplasty Balloon angioplasty Pressure Values from Cardiac Catheterization

 Systemic Arterial Pressure 60 – 90/20 – 60 mm Hg (birth to 5 days)  R atrial pressure 3 mm Hg  R vent. Pressure 30/3 mm Hg  Pulmonary Wedge Pressure 6 – 10 mmHg  L atrial pressure 8 mm Hg  L ventricular pressure 100/6 mm Hg erex Self Study the following Congenital Heart Defects Congenital Heart Defects

Defects with INCREASED pulmonary blood flow  PDA  VSD  ASD  AV Canal

Obstructive defects with pulmonary venous congestion  COA  Aortic stenosis Congenital Heart Defects

Obstructive defects with DECREASED pulmonary blood flow  TOF  Pulmonary stenosis  Pulmonary atresia  Tricuspid atresia

Mixed Defects  TGA  TA  TAPVR  HLHS Ventricular Septal Defect

 VSDs are most common form of all CHDs  Presentation and management will vary according to size of VSD  50% - 75% of small defects will close spontaneously; 20% of large defects will close independently VSD PVR

Pulmonary artery SVR hypertrophy stenosis Left Right Shunt Pulmonary Edema Pulmonary Blood Flow Moderate VSD VSD • Fatigue with feeding • Recurrent respiratory infections Large VSD • Loud, blowing pansystolic murmur @ LLSB • CXR: increased Small VSD pulmonary vascular  Asymptomatic markings, cardiomegaly  High - pitched pansystolic murmur @ LSB 4 - 10 • @ 1 - 2 MOL: CHF, DOL hepatomegaly, recurrent respiratory infections Atrial Septal Defect

 Female to Male 2:1  Spontaneous closure around 20%  Defect can be in ostium primum or secundum (most common)  PFO ASD ASD

 Symptoms present in 50% of patients IF ID’d in infancy (most go unrecognized)  Failure to thrive  Recurrent respiratory infections  Systolic murmur @ 2 nd ICS LSB, persistent split S2 if defect is large, diastolic murmur @ LLSB AV Canal

♥ Endocardial Cushion Defect or Atrioventricular Septal Defect

♥ Partial involves the atria

♥ Complete involves atria and ventricles

♥ Left to right shunt AV canal Risk Factors

Trisomy 21 Rubella or other viral illness during early pregnancy Alcohol consumption Poorly controlled diabetes Smoking Parent with a CHD AV canal complications ♥ Cardiomegaly

♥ On auscultation, thrill at the LLSB. Loud pansystolic murmur at LLSB radiating to back

♥ Pulmonary Hypertension

♥ Respiratory Tract Infections

♥ Congestive Heart Failure Complications after correction of AV canal

♥ Regurgitant heart valves

♥ Stenosis of the heart valves

♥ Arrythmias

♥ PPHN

♥ Breathing difficulties associated with damage COA – Coarctation of the aorta

♥ Juxtaductal narrowing of aorta

♥ Frequently associated

♥ Often associated with hypoplastic transverse arch

♥ Mild left heart hypoplasia COA - Coarctation of the aorta ♥ As the ductus arteriosus closes, the area of coarctation narrows

♥ The result is decreased systemic blood flow, pulmonary venous congestion

♥ In severe cases, CHF results from decreased left ventricular function

♥ If severe, w ith ductal closure, shock and tissue hypoxia COA

 Male dominance 2:1  Associated with Turner Syndrome

Presentation: • Decreased or absent LE pulses • Higher in UE; BPs may wax and wane with bidirectional flow through PDA • “systolic thrill @ suprasternal notch” • Cardiomegaly + increased pulmonary vascular markings on CXR COA Management

 Treat/Man age CHF

 PGE

 Surgery Aortic Stenosis

 4 x’s more likely in males  3 types: Valvar is the most common  If AS is severe in utero, fetus will develop LV hypoplasia and L - sided syndrome Aortic stenosis

 Valvular aortic stenosis has a bicuspid aortic valve  LV is hypertrophied Aortic Stenosis Presentation

 If not diagnosed in utero and not severe, asymptomatic at birth , then progresses to CHF  Harsh systolic murmur in URSB that radiates  CXR: cardiomegaly but normal pulmonary vascular markings Aortic Stenosis: Treatment

Balloon Valvuloplasty TOF – Tetralogy of Fallot

♥ Most common CYANOTIC heart defect

4 anomalies  Large VSD  Right ventricular outflow tract obstruction  Overriding aorta  Right ventricular hypertrophy

♥ Symptom severity depends on the degree of right ventricular outflow tract obstruction TOF TOF Presentation

 Harsh systolic murmur with thrill @ ULSB  Boot shaped heart on CXR TOF - Management

♥ Supplemental FiO2 to treat cyanosis

♥ The majority are not ductal dependent

♥ If saturation still below 75% on oxygen may have significant pulmonary stenosis or atresia and need PGE TET Spell Hypoxic Spell

♥ Cyanotic or hypercyanotic spell  Progressive hypoxia  Hyperpneic  Pale, flaccid  Immediate treatment needed  Organ damage if severe hypoxia and acidosis  Eventual loss of consciousness

TET Spell Management

Calm infant Increase systemic vascular resistance to decrease the right to left shunt at the VSD Knees to chest May need to be sedated FiO2 but will only improve oxygenation once right to left shunt is decreased May need intubation and correction of metabolic acidosis Pulmonary stenosis

♥ Stenosis is a narrowing

♥ Varying degrees of narrowing; severity of symptoms depends on degree of narrowing

♥ Most common form is valvular Mild Pulmonary Stenosis

 Loud systolic murmur at LUSB is the only clinical finding Severe Pulmonary Stenosis

♥ If critical PS, the right ventricle cannot eject sufficient blood flow to the pulmonary artery to maintain normal oxygen saturations.

♥ If critical, PGE is required to allow blood to shunt left to right to perfuse the

♥ A newborn with critical pulmonary stenosis and intact VSD presents an emergency situation that requires immediate treatment, either balloon dilation of the valve or surgery. Pulmonary Atresia with VSD PA Pulmonary Atresia with intact ventricular septum

♥ Atresia means not formed, so absence ♥ Atretic pulmonary valve ♥ Hypertrophied right ventricle ♥ Hypoplastic right ventricle ♥ Blood flow R to L across PFO ♥ Pulmonary blood flow is dependent on the PDA ♥ Tricuspid Atresia

 Agenesis of the tricuspid valve  VSD often present  Hypoplastic RV  Pulmonary valve atresia or stenosis  TGA in 30% of cases of tricuspid atresia HLHS – hypoplastic left heart syndrome

♥ Hypoplasia of left ventricle

♥ Severe mitral valve stenosis or atresia or severe aortic valve stenosis or atresia

♥ Hypoplastic ascending aorta and transverse arch

♥ Coarctation is a frequent finding HLHS Ductal dependent blood flow right to left to perfuse all regions of the body Management : HLHS

♥ To open PDA and to improve systemic perfusion

♥ Intubation and ventilation to reduce work of breathing and strain on the heart

♥ Avoid hyperoxia and hypocarbia (both will decrease PVR at the expense of systemic blood flow)

♥ Inotropic support may improve myocardial function

♥ Follow arm and leg BPs TGA – Transposition of the Great Arteries Great arteries are transposed relative to the ventricles TGA

♥ Circulation pattern is parallel ♥ The majority of the blood from each ventricle is circulated back to the same ventricle ♥ Mixing must occur: Best to have a VSD and a PFO/ASD that is not restrictive Use PGE to open the PDA ♥ If no VSD and a restrictive PFO/ASD a Rashkind /Balloon septostomy may be needed

There needs to be adequate mixing at 2 sites! TAPVR – Total Anomalous Pulmonary Venous Return TAPVR Types

♥ Supracardiac emptying into the left vertical (most common type 80 - 90%) which then drains into the SVC

♥ Cardiac emptying into the coronary sinus or right atrium

♥ Infradiaphragmatic emptying into the vertical vein that descends through the diaphragm into the portal vein or IVC Infracardiac Obstructed TAPVR

♥ Oxygenated pulmonary blood mixes with deoxygenated systemic blood and returns to the right atrium to shunt right to left across the PFO/ASD to get to the left atrium and eventually to the body

♥ Severe hypoxia and profoundly ill after birth Management Infracardiac Obstructed TAPVR

- Intubation 100% FiO2

- Treat acid /base disturbance

- Treat hypotension, hypothermia

- Emergency corrective surgery is required to anastomose the pulmonary to the heart CXR Cardiac or supracardiac CXR Obstructed TAPVR CXR can be confused with RDS or PNA Truncus Arteriosus

 One great vessel arising from both ventricles, VSD  One vessel supplying systemic and pulmonary circulation  As PVR decreases, shunting occurs L to R across VSD overloading lungs and LV (unless PS) Truncus Arteriosus

 Bounding pulses, widened pulse pressure  Severe cyanosis if TA with PS  Harsh systolic murmur LLSB, systolic ejection click, single S2  Management: Treat CHF (medications include diuretics, digoxin and ACE inhibitors)  Surgical repair Sample Cardiac Questions

Normal values for pulmonary artery pressures in a term infant would be: A. RAP 7 - 10 mm Hg, LAP 5 - 7 mm Hg and PAOP 4 mm Hg B. RAP 3 mm Hg, LAP 8 mm Hg and PAOP 6 - 10 mm Hg C. RAP 10 - 20 mm Hg, LAP 12 - 14 mm Hg and PAOP 10 - 14 mm Hg The type of murmur commonly heard in patients with tricuspid atresia is: A. A diastolic murmur B. A systolic murmur C. A holosystolic murmur An ASD results in: A. No damage to the septum B. A left - to - right shunt with volume overload C. A diastolic murmur at the USB Neonates with cyanotic heart defects are at particular risk for: A. Intrarenal kidney injury B. CHF C. Polycythemia A mechanism by which a newborn can respond to increased volume is via: A. A baroreceptor stretch B. An increased heart rate C. A decreased PVR During your assessment, you note that the R brachial is stronger than the L. You conclude: A. Cardiac tamponade B. Thoracic anuerysm C. Supravalvular aortic stenosis