Cardiac+Round+2

//**Defects with decreased pulmonary blood flow, cyanotic disorders:**// Pulmonary Stenosis Tetralogy of Fallot Transposition of the Great Arteries Tricuspid Atresia
 * //Cardiac Round 2// **

**Pathology:** The amount of blood to the pulmonary artery is decreased Shunting of unoxygenated blood from the right side to the left Mixing of oxygenated and unoxygenated Infants are hypoxic and cyanotic Polycythemia (^ hematocrit) Erythrocytosis (red cell production increases) Increased blood viscosity Increases ventricular work

**Pulmonary stenosis:** <span style="color: #e23232; font-family: 'Times New Roman',Times,serif; font-size: 180%;"> <span style="font-family: 'Times New Roman',Times,serif; font-size: 120%;">Narrowing of the pulmonary value <span style="font-family: 'Times New Roman',Times,serif; font-size: 120%;">This obstructs blood flow from the right ventricle to the lungs <span style="font-family: 'Times New Roman',Times,serif; font-size: 120%;">Incidence - 8-10%

<span style="color: #e23232; font-family: 'Times New Roman',Times,serif; font-size: 140%;">Manifestations: <span style="font-family: 'Times New Roman',Times,serif; font-size: 120%;">Mild to moderate can be asymptomatic <span style="font-family: 'Times New Roman',Times,serif; font-size: 120%;">Severe – dyspnea with exertion, fatigue, cyanosis <span style="font-family: 'Times New Roman',Times,serif; font-size: 120%;">Murmur

<span style="color: #e23232; font-family: 'Times New Roman',Times,serif; font-size: 140%;">Treatment: <span style="font-family: 'Times New Roman',Times,serif; font-size: 120%;">Valvuloplasty during cardiac cath <span style="font-family: 'Times New Roman',Times,serif; font-size: 120%;">Surgery valvotomy

<span style="color: #e23232; font-family: 'Times New Roman',Times,serif; font-size: 180%;">**Tetralogy of Fallot** <span style="font-family: 'Times New Roman',Times,serif; font-size: 120%;">4 components <span style="font-family: 'Times New Roman',Times,serif; font-size: 120%;">VSD <span style="font-family: 'Times New Roman',Times,serif; font-size: 120%;">Pulmonary stenosis <span style="font-family: 'Times New Roman',Times,serif; font-size: 120%;">Right ventricular hypertrophy <span style="font-family: 'Times New Roman',Times,serif; font-size: 120%;">An overriding aorta <span style="font-family: 'Times New Roman',Times,serif; font-size: 120%;">9-14% of cases of heart disease, most common cyanotic heart defect

<span style="font-family: 'Times New Roman',Times,serif; font-size: 120%;">Right ventricle hypertrophied because of pumping against resistance at the stenosis <span style="font-family: 'Times New Roman',Times,serif; font-size: 120%;">Of the pulmonary artery

<span style="color: #e23232; font-family: 'Times New Roman',Times,serif; font-size: 140%;">Manifestations: <span style="font-family: 'Times New Roman',Times,serif; font-size: 120%;">Vary depending on degree of malformation <span style="font-family: 'Times New Roman',Times,serif; font-size: 120%;">Profoundly cyanotic or not <span style="font-family: 'Times New Roman',Times,serif; font-size: 120%;">Tet spells result during exertion such as feeding, crying, defecating <span style="font-family: 'Times New Roman',Times,serif; font-size: 120%;">Result of decrease in pulmonary blood flow

<span style="color: #e23232; font-family: 'Times New Roman',Times,serif; font-size: 140%;">Diagnosis: <span style="font-family: 'Times New Roman',Times,serif; font-size: 120%;">Echo

<span style="color: #e23232; font-family: 'Times New Roman',Times,serif; font-size: 140%;">Treatment: <span style="font-family: 'Times New Roman',Times,serif; font-size: 120%;">Surgical intervention

<span style="color: #e23232; font-family: 'Times New Roman',Times,serif; font-size: 140%;">Nursing Interventions: <span style="font-family: 'Times New Roman',Times,serif; font-size: 120%;">Knee-chest position for tet spells, squatting <span style="font-family: 'Times New Roman',Times,serif; font-size: 120%;">Supplemental oxygen <span style="font-family: 'Times New Roman',Times,serif; font-size: 120%;">Decrease blood viscosity <span style="font-family: 'Times New Roman',Times,serif; font-size: 120%;">Surgical repair at 3-12 months

<span style="color: #e23232; font-family: 'Times New Roman',Times,serif; font-size: 180%;">**Transposition of the Great Arteries:** <span style="font-family: 'Times New Roman',Times,serif; font-size: 120%;">Great vessels reversed <span style="font-family: 'Times New Roman',Times,serif; font-size: 120%;">Incidence 7-8% <span style="font-family: 'Times New Roman',Times,serif; font-size: 120%;">Parallel circulation with no oxygenated blood getting to the system <span style="font-family: 'Times New Roman',Times,serif; font-size: 120%;">Only chance for survival is the PDA, ASD, PFO <span style="font-family: 'Times New Roman',Times,serif; font-size: 120%;">Prostaglandin therapy

<span style="color: #e23232; font-family: 'Times New Roman',Times,serif; font-size: 140%;">Manifestations: <span style="font-family: 'Times New Roman',Times,serif; font-size: 120%;">Cyanosis not responsive to oxygen <span style="font-family: 'Times New Roman',Times,serif; font-size: 120%;">Appear otherwise healthy

<span style="color: #e23232; font-family: 'Times New Roman',Times,serif; font-size: 140%;">Diagnosis: <span style="font-family: 'Times New Roman',Times,serif; font-size: 120%;">ECHO <span style="font-family: 'Times New Roman',Times,serif; font-size: 120%;">Sometimes cardiac cath.

<span style="color: #e23232; font-family: 'Times New Roman',Times,serif; font-size: 140%;">Treatment: <span style="font-family: 'Times New Roman',Times,serif; font-size: 120%;">Stabilization <span style="font-family: 'Times New Roman',Times,serif; font-size: 120%;">Prostaglandin therapy <span style="font-family: 'Times New Roman',Times,serif; font-size: 120%;">Atrial septostomy

<span style="color: #e53434; font-family: 'Times New Roman',Times,serif; font-size: 23.399999618530273px;">//**Defects that obstruct left ventricular outflow**// <span style="color: #e53434; font-family: 'Times New Roman',Times,serif; font-size: 18.200000762939453px;">Coarctation of the Aorta <span style="color: #e53434; font-family: 'Times New Roman',Times,serif; font-size: 18.200000762939453px;">Aortic Stenosis <span style="color: #e53434; font-family: 'Times New Roman',Times,serif; font-size: 18.200000762939453px;">Hypoplastic left heart syndrome

<span style="color: #e53434; font-family: 'Times New Roman',Times,serif; font-size: 18.200000762939453px;">Manifestations <span style="font-family: 'Times New Roman',Times,serif; font-size: 15.600000381469727px;">Decreased cardiac output – shock, acidosis, death <span style="font-family: 'Times New Roman',Times,serif; font-size: 15.600000381469727px;">Older child asymptomatic, upper extremity hypertension, diminished pulses in the lower extremity

<span style="color: #e53434; font-family: 'Times New Roman',Times,serif; font-size: 18.200000762939453px;">Diagnosis: <span style="font-family: 'Times New Roman',Times,serif; font-size: 15.600000381469727px;">Echo <span style="font-family: 'Times New Roman',Times,serif; font-size: 15.600000381469727px;">MRI

<span style="color: #e53434; font-family: 'Times New Roman',Times,serif; font-size: 18.200000762939453px;">Treatment <span style="font-family: 'Times New Roman',Times,serif; font-size: 15.600000381469727px;">Surgical <span style="font-family: 'Times New Roman',Times,serif; font-size: 15.600000381469727px;">Balloon dilatation <span style="font-family: 'Times New Roman',Times,serif; font-size: 15.600000381469727px;">Control hypertension

<span style="color: #e53434; font-family: 'Times New Roman',Times,serif; font-size: 23.399999618530273px;">**Coarctation of the Aorta** <span style="font-family: 'Times New Roman',Times,serif; font-size: 15.600000381469727px;">Narrowing of the aorta most commonly across from the PDA <span style="font-family: 'Times New Roman',Times,serif; font-size: 15.600000381469727px;">Incidence 8-11% CHD

<span style="color: #e53434; font-family: 'Times New Roman',Times,serif; font-size: 18.200000762939453px;">Pathology:
 * <span style="font-family: 'Times New Roman',Times,serif; font-size: 15.600000381469727px;">Narrowing of the aorta, which sends blood to the body.
 * <span style="font-family: 'Times New Roman',Times,serif; font-size: 15.600000381469727px;">Like a kink in a garden hose pressure builds up on the other side, the aortic ‘kink’ causes left ventricular hypertrophy (enlargement) as it pushes against the pressure

<span style="color: #e53434; font-family: 'Times New Roman',Times,serif; font-size: 18.200000762939453px;">Clinical manifestations: <span style="color: #e53434; font-family: 'Times New Roman',Times,serif; font-size: 16.899999618530273px;">***Blood pressures in the upper extremities are higher than the lower extremities (should be approximately the same)***

<span style="color: #e53434; font-family: 'Times New Roman',Times,serif; font-size: 18.200000762939453px;">Diagnosis: <span style="font-family: 'Times New Roman',Times,serif; font-size: 15.600000381469727px;">Examination <span style="font-family: 'Times New Roman',Times,serif; font-size: 15.600000381469727px;">Echo <span style="font-family: 'Times New Roman',Times,serif; font-size: 15.600000381469727px;">MRI possibly for more details

<span style="color: #e53434; font-family: 'Times New Roman',Times,serif; font-size: 18.200000762939453px;">Treatment: <span style="font-family: 'Times New Roman',Times,serif; font-size: 15.600000381469727px;">Relief of obstruction by balloon dilatation or surgery (which cuts out the kink and reattaches the ends) <span style="font-family: 'Times New Roman',Times,serif; font-size: 15.600000381469727px;">Surgery at 3-5 years of age so it will not reoccur <span style="font-family: 'Times New Roman',Times,serif; font-size: 15.600000381469727px;">Medications utilized are medications for hypertension

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<span style="color: #e53434; font-family: 'Times New Roman',Times,serif; font-size: 23.399999618530273px;">**Aortic Stenosis –** <span style="font-family: 'Times New Roman',Times,serif; font-size: 15.600000381469727px;">Narrowing of the aortic valve <span style="font-family: 'Times New Roman',Times,serif; font-size: 15.600000381469727px;">Incidence 3-6%

<span style="color: #e53434; font-family: 'Times New Roman',Times,serif; font-size: 18.200000762939453px;">Pathology: <span style="font-family: 'Times New Roman',Times,serif; font-size: 15.600000381469727px;">Increased ventricular work, enlargement (hypertrophy)

<span style="color: #e53434; font-family: 'Times New Roman',Times,serif; font-size: 18.200000762939453px;">Manifestations: <span style="font-family: 'Times New Roman',Times,serif; font-size: 15.600000381469727px;">Diagnosis usually not made until the child is older unless severe <span style="font-family: 'Times New Roman',Times,serif; font-size: 15.600000381469727px;">Murmur <span style="font-family: 'Times New Roman',Times,serif; font-size: 15.600000381469727px;">Exertional dyspnea <span style="font-family: 'Times New Roman',Times,serif; font-size: 15.600000381469727px;">Angina

<span style="color: #e53434; font-family: 'Times New Roman',Times,serif; font-size: 23.399999618530273px;">**Hypoplastic Left Heart Syndrome:** <span style="font-family: 'Times New Roman',Times,serif; font-size: 15.600000381469727px;">Lack of development of the left ventricle (pump) which circulates oxygenated blood to the body through the aorta.

<span style="font-family: 'Times New Roman',Times,serif; font-size: 15.600000381469727px;">

<span style="color: #e53434; font-family: 'Times New Roman',Times,serif; font-size: 18.200000762939453px;">Manifestations: <span style="font-family: 'Times New Roman',Times,serif; font-size: 15.600000381469727px;">Cyanotic <span style="font-family: 'Times New Roman',Times,serif; font-size: 15.600000381469727px;">Cardiovascular collapse, hypotension, tachycardia, cyanosis, tachypnea

<span style="color: #e53434; font-family: 'Times New Roman',Times,serif; font-size: 18.200000762939453px;">Diagnosis: <span style="font-family: 'Times New Roman',Times,serif; font-size: 15.600000381469727px;">Echo

<span style="color: #e53434; font-family: 'Times New Roman',Times,serif; font-size: 18.200000762939453px;">Treatment: <span style="font-family: 'Times New Roman',Times,serif; font-size: 15.600000381469727px;">Used to be inoperable, comfort measures until death <span style="font-family: 'Times New Roman',Times,serif; font-size: 15.600000381469727px;">Died after the PDA closed <span style="color: #e53434; font-family: 'Times New Roman',Times,serif; font-size: 16.899999618530273px;">Presently 3 options
 * <span style="color: #e53434; font-family: 'Times New Roman',Times,serif; font-size: 16.899999618530273px;">No treatment
 * <span style="color: #e53434; font-family: 'Times New Roman',Times,serif; font-size: 16.899999618530273px;">Cardiac transplantation
 * <span style="color: #e53434; font-family: 'Times New Roman',Times,serif; font-size: 20.280000686645508px;">Palliative surgery <span style="font-family: 'Times New Roman',Times,serif; font-size: 15.600000381469727px;">, prostaglandins, O2 <21%, or CO2 causes vasoconstriction of pulmonary
 * <span style="font-family: 'Times New Roman',Times,serif; font-size: 15.600000381469727px;">O2 sat levels ~ 75%

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Survivors:[|Hypoplastic Heart Survivors]

<span style="color: #bc1a1a; font-family: 'Times New Roman',Times,serif; font-size: 18px;">References:

<span style="font-family: 'Times New Roman',Times,serif; font-size: 14pt;">O'Brien, P., & Baker, A. L. (2009). Chapter 25: The child with cardiovascular dysfunction. In M. J. Hockberry & D. Wilson (Eds.), Wong's essentials of pediatric nursing (8th ed., pp. 861-910). St. Louis, MO: Mosby Elsevier.

<span style="color: #bc1a1a; font-family: 'Times New Roman',Times,serif; font-size: 17px;">Pictures/Videos: <span style="font-family: 'Times New Roman',Times,serif; font-size: 16.899999618530273px;">Stenosis pictures located at heartdefectsforeveryone.blogspot.com

<span style="font-family: 'Times New Roman',Times,serif; font-size: 16.899999618530273px;">Tetralogy of fallot video located at

<span style="font-family: 'Times New Roman',Times,serif; font-size: 16.899999618530273px;">Transposition of the Great Vessels videos located at:

<span style="font-family: 'Times New Roman',Times,serif; font-size: 15.600000381469727px;">Coarctation of the aorta image located at mykentuckyheart.com

<span style="font-family: 'Times New Roman',Times,serif; font-size: 15.600000381469727px;">Hypoplastic left heart syndrome image located at <span style="font-family: 'Times New Roman',Times,serif; font-size: 15.600000381469727px;">http://www.pediatrics.wisc.edu/research/research-groups/hokanson/congenital-heart-diseases.html

<span style="font-family: 'Times New Roman',Times,serif; font-size: 15.600000381469727px;">WYFF4 Left Hypoplastic Heart Interview Emersion Rose Smith http://youtu.be/haRrrq9gVdI