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Neonatal & Pediatrics > 11- Cardiac Defects > Flashcards

11- Cardiac Defects Flashcards

1
Q

Acyanotic heart defects

A

-Any structural abnormality that does not cause right-tot-left intra-cardiac shunting
-Pt should have normal O2 sats

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2
Q

Types of Acyanotic heart defects

A

-Septal wall defects (2) (holes in septum of hear that vary in location)
-Aortic stenosis
-Pulmonic stenosis
-Coarctation of the aorta
-Double aortic arch

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3
Q

Septal wall defects

A

-Atrial septal defects (ASD)
-Ventricular septal defects (VSD)
-Represent the 2 most common types of (CHD)

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4
Q

Atrial septal defects (ASD)

A

-Opening in the intra-atrial septum- This creates an anatomical connection between 2 uppermost chambers of the heart
-Rarely cause symptoms in infancy- undiagnosed
-Forms when the fusion of septum primum and septum secundum occurs.

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5
Q

Clinical manifestations of ASD

A

-Increased workload on the right ventricle
-Increased blood flow to the lungs
-These are secondary to a large defect >6mm in diameter and only if symptoms are present

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6
Q

Symptoms of ASD

A

-Fatigue
-Exercise intolerance
-Failure to thrive- not growing
-Poor weight gain
-Tachypnea

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7
Q

How to diagnose ASD?

A

Echocardiogram

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8
Q

Management and treatment of ASD

A

-No symptoms: Managed by a cardiology clinic for years
-Small ASD <6mm in diameter: Usually spontaneously close by the age of 2
-ASD>6mm: Usually require closure prior to the child starting school

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9
Q

Indication for closure includes….

A

-Symptoms of heart failure
-Pulmonary hypertension
-Risk or history of paradoxical embolus
-History of arrhythmia

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10
Q

Ventricular Septal Defects (VSD)

A

-Most common congenital heart lesion
-Causes a connection btw right and left ventricle
-Can occur as a single lesion or with other heart defects

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11
Q

Other defects VSD can occur with

A

-Tetralogy of Fallot (TET)
-Pulmonary atresia
-Complete atrioventricular canal
-Transposition of the great arteries (TGA)
-Patent ductus arteriosus (PDA)

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12
Q

Clinical Manifestations of VSD

A

-Small defect with little intra-cardiac shunting will usually remain asymptomatic
-With larger defects:
—Increased pulmonary blood flow
—Increased pulmonary venous return to the left side of the heart
—Subsequent left ventricular volume overload.

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13
Q

Symptoms of VSD

A

-Tachypnea
-Poor feeding
-Failure to thrive
-Sweating
-Irritability
-Auscultation of the lungs may reveal rales secondary to pulmonary edema.

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14
Q

How do you diagnose VSD?

A

-Echocardiogram

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15
Q

Management and treatment of VSD

A

-Asymptomatic requires none
-With symptoms: Diuretics, Systemic afterload reduction (ACE inhibitors and Inotrops)
-Pts with failure to thrive may need a higher calorie formula to accommodate for increased caloric need

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16
Q

Surgical indications for VSD

A

-Heart failure despite medical management
-PPHN- persistent pulmonary hypertension
-Subpulmonic or membranous VSD with aortic valve regurgitation

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17
Q

Atrioventricular septal defects

A

-Spectrum of abnormalities that involve defects in the portion of the atrial and/or ventricular septum directly adjacent to the atrioventricular valves (tricuspid and mitral).
-Common in children with Trisomy 21 (Down syndrome).

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18
Q

3 types of atrioventricular septal defects

A

-Partial
-Transitional
-Complete

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19
Q

Treatment of atrioventricular septal defects

A

-Asymptomatic: Treated similarly as those with VSD
-Definitive therapy involves surgical repair

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20
Q

Aortic stenosis

A

-Any discrete narrowing that occurs btw the left ventricle and the aorta
-Most common cause: Bicuspid aortic valve (Aortic valve consists of 2 functional leaflets instead of 3)

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21
Q

Severity of aortic stenosis depends on

A

-The degree of obstruction, which is a calculated measurements from an ECHOCARDIOGRAM

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22
Q

Aortic stenosis severity

A

-Mild: <25 mmHg gradient
-Moderate: 25-50 mmHg gradient
-Severe: 50mmHg gradient. CO is usually maintained. There is an increase workload on the left ventricle and myocardial ischemia can develop

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23
Q

Symptoms of aortic stenosis

A

Infants: Tachypnea, poor feeding, growth failure
Children: Syncope, progressive exercise intolerance, fatigue, and chest pain

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24
Q

How can you diagnose aortic stenosis?

A

Echocardiogram

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25
Q

Management and treatment of aortic stenosis

A

-Therapy depends on severity
-Neonates with shock: Placed on PGEI to help ductus open to improve circulation
-Mild to moderate: Outpatient cardiology with serial echocardiograms
-Sever: Reduce obstruction and create an exit of blood across the left ventricular outflow tract

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26
Q

Surgeries for aortic stenosis

A

-Valvuloplasty: A balloon tip is passed into the valve orifice and inflated to enlarge the opening
-Valvotomy: Division of the valve leaflets of valve replacement

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27
Q

What happens when aortic stenosis is left untreated?

A

-Risk of sudden cardiac death
-Infective endocarditis
-Inflammation of heart valves

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28
Q

Pulmonic stenosis

A

-A narrowing in the right ventricular outflow tract between the right ventricle and the main pulmonary artery.
-It results from abnormally formed valve leaflets that may be dysplastic or form an abnormal dome shape.
-Can result form obstruction in the R ventricular outflow tract: increases the workload on R ventricle, which can cause right ventricular hypertrophy

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29
Q

Classifications of Pulmonic stenosis

A

-Mild: no symptoms
-Moderate to sever: Increase R ventricular afterload.
——Dyspnea on exertion
——Fatigue
——Cyanosis

30
Q

How to diagnose pulmonic stenosis?

A

Echocardiogram- Look at anatomy, size and degree of obstruction

31
Q

Treatment for pulmonary stenosis

A

-Mild: no therapy
-Moderate: Transcatheter ballon valvuloplasty, surgical valvotomy, or valve replacement

32
Q

Coarctation of the aorta

A

-A discrete narrowing of the descending thoracic aorta, usually distal to the takeoff of the left subclavian artery.
-Involves the extension of a shelf-like structure into the lumen of the aorta.
-The “shelf” represents thickening of the first two layers of the aortic vessel. (Intima and Media)

33
Q

Clinical manifestation of coarctation of aorta

A

-Obstruction to blood in descending aorta. Blood is diverted to brachiocephalic, left carotid, and left subclavian artery (Results in difference in BP btw upper and lower extremities)
-Upper extremity systolic blood pressure is at least 10 mm Hg higher than lower extremities.

34
Q

Symptoms of coarctation of aorta

A

-Older patients may be asymptomatic.
-Diminished or absent femoral pulses
-Brachio-femoral delay
-Continuous flow murmur

35
Q

Diagnosis of coarctation of aorta

A

-Echocardiogram
-MRI

36
Q

Treatment of coarctation of aorta

A

-Surgical repair: Coarctation segment is removed by the surgeon, and the aorta is reconnected in an end-to-end fashion.
-Alternative: Dilation and widening of the narrowed blood vessel

37
Q

Patients with coarctation of aorta are susceptible to…

A

-Persistent hypertension
-Vocal cord paresis or paralysis
-Chylothorax- digestive system lymph accumulates in chest cavity
-Lower extremity paralysis
-Post-coarctectomy syndrome- GI stuff

38
Q

Double aortic arch

A

-During the development of the fetus, six symmetrical arches undergo remodeling to form the aortic arch and its major branches. (innomiate artery, left common carotid artery, left subclavian artery)
-Occurs when one of the arches fails to remodel correctly and is included in the class of anomalies.

39
Q

Double aortic arch is also known as…

A

-Complete vascular ring
——Encircles and causes compression of both the esophagus and trachea.
——Instead of a single left-sided arch, patients have an arch that bifurcates into a right and left aortic arch.

40
Q

Clinical manifestations of double aortic arch

A

-Adjacent to esophagus: Feeding difficulty, Gastroesophageal reflux (GERD), Failure to thrive, and Dysphagia.
-Adjacent to trachea: Intrathoracic upper airway obstruction

41
Q

Signs and symptoms of double aortic arch

A

-Cough
-Wheezing
-Stridor
-Persistent infections
-Critical airway obstruction

42
Q

Diagnosis of double aortic arch

A

-Barium swallow
-CT angiography
-MRI

43
Q

Management and treatment of double aortic arch

A

-Management depends on symptoms
-Severe dysphasia: nasogastric tube feeds
-Sever upper airway obstruction: intubation and MV (PEEP helps stent open airway structures being compressed by anterior aortic arch)
-Child is diagnosed: Should be immediately referred for surgical repair

44
Q

Surgical interventions for double aortic arch

A

-The nondominant left aortic arch division and ligation, which leaves the dominant right aortic arch intact. This relieves airway obstruction, however, mild airway symptoms can still be present.

45
Q

Cyanotic heart defects

A

Result from structural abnormalities that lead to significant mixing of oxygenated and deoxygenated blood and cause oxygen saturations less that 85%.

46
Q

Types of cyanotic heart defects

A

-Tetralogy of Fallot
-Total anomalous pulmonary venous return
-Transposition of the great arteries
-Hypoplastic left heart syndrome
-Truncus arteriosus

47
Q

Any neonate presenting with O2 sats of 85% to 90% should be assessed for what?

A

To determine whether the cause of hypoxemia is lung disease or CHD

48
Q

Measures to determine cardiac defects in neonates

A

-Chest radiograph
-Electrocardiograph
-Hyperoxia test
-Echocardiogram
-Auscultation (may reveal abnormal heart sounds)

49
Q

Hyperoxia test

A

-ABG are obtained on room air and FiO2 100% and compared to assess the pt’s ability to oxygenate.
-Baseline: R radial ABG will give accurate PaO2, directly from the aorta, prior to PDA
-100% FiO2 15 minutes later: PaO2 is assessed

50
Q

Hyperoxia test PaO2 results from 100% FiO2

A

-PaO2 >150mmHg: hypoxemia is related to lung disease
-PaO2 <150mmHg: Cyanotic heart defect should be suspected. Low PaO2 is due to shunting and mixing of oxygenated and deoxygenated blood.

51
Q

Prostaglandin Infusion

A

Prostaglandin E is given to help with cyanosis.
-Prostaglandin E are hormone-like substances that maintain patency of the PDA.
-May be discontinued after the specific cardiac lesion has been identified

52
Q

Tetralogy of Fallot

A

-A combination of four conditions
—-VSD
—-An aorta that overrides the VSD
—-Obstruction of the right ventricular outflow tract (RVOTO)
—-Right ventricular hypertrophy
-The most common form of cyanotic CHD

53
Q

Tet spell

A

A sudden increase in RVOTO can cause a period of hypercyanosis.

54
Q

Treatment for a tet spell

A

-O2
-Knee to chest manuevers
-Medications ton increase SVR (Phenylephrine)
-Volume administration (increase flow into pulmonary vasculature)
-Sedatives (Morphine, Decrease catecholamines, Decrease spasm at the opening of the pulmonary valve).

55
Q

Symptoms of tet spells

A

Mild obstruction: May have no symptoms
Severe RVOTO: Severe pulmonary stenosis or pulmonary atresia

56
Q

Treatment of tet spell

A

Severe symptoms: Early treatment to provide pulmonary blood flow: PGE1 infusion, Early surgical palliation, and Repair

57
Q

Total anomalous pulmonary venous return (TAPVR)

A

Results when there is no connection between the pulmonary vein confluence (the location where the pulmonary veins come together) and the left atrium.

58
Q

TAPVR is life-threatening because

A

-No blood delivery into the left side of the heart
-Impossible for circulation of oxygenated blood to the body without communication between the right and left sides of the heart (Septal defect)

59
Q

There are 4 types of TAPVR

A

-Supracardiac TAPVR
-TAPVR to the coronary sinuses
-Infracardiac TAPVR
-Mixed-type TAPVR
-The only difference between these types is the location of the TAPVR

60
Q

Symptoms of TAPVR

A

-Asymptomatic at birth
-Cyanosis is often not apparent
-Tachypnea and Feeding difficulties (Develop over the first few weeks of life)
-Failure to thrive.

61
Q

Transposition of the great arteries (TGA)

A

Two main arteries leaving the heart have changed places, so that the aorta arises from the right ventricle and the pulmonary artery arises from the left ventricle.

62
Q

Common with TGA

A

-VSD
-Left ventricular outflow tract obstruction
-Aortic abnormalities (rare)

63
Q

Symptoms of TGA

A

If there is inadequate mixing across an associated intracardiac or extracardiac shunt, a neonate with TGA will present with rapidly progressive cyanosis within the first few hours of life.

64
Q

Diagnosis of TGA

A

-Rapid diagnosis and emergent intervention are required for survival
-ECHOCARDIOGRAM
-Physical exam is non specific (No cardiac murmur. Unless there is no VSG present; Electrocardiogram is normal; Chest radiograph is often unremarkable.)

65
Q

Treatment of PGA

A

-Intracardiac shunt needs to be created immediately in order for the neonate to survive.
-Atrial septostomy- creates a hole in the atrial wall (wide-open-atrial-level shunt allows mixing to occur and sats to stabilize)
-Atrial switch- surgical repair for TGA

66
Q

Hypoplastic left heart syndrome (HLHS)

A

-Cyanotic heart disease in which parts of the left side of the heart do not fully develop.
-Left ventricle: small and unable to sustain systemic circulation, small and non-apex forming.
-Left atrium: small in since d/t lack of flow
-Aorta: small until PDA, where flow from the PDA supplies the normal-size descending aorta
-Includes an atrial septal defect, which supplies oxygenated blood flow to the right heart.

67
Q

Staged repair for HLHS

A

-Norwood: Combine aorta and pulmonary artery using a shunt
-Bidirectional Glen: Connection of superior vena cava to pulmonary artery, and removal of pulmonary artery shunt.
-Fontan: Connection of inferior vena cava to pulmonary artery

68
Q

Hypoxic gas delivery

A

Used for pts who consistently saturate>85% to increase PVR to maintain appropriate systemic blood flow prior to stage 1 repair
-Nitrogen is mixed with room air to achieve an FIO2 of 0.15 to 0.20.
-FIO2 < 0.15 can put patients at risk for tissue hypoxia.
-Delivery devices include: nasal cannula, oxyhood, or ventilator.
-An external oxygen analyzer should be used to ensure the appropriate FIO2 is delivered.

69
Q

Truncus Arteriosus

A

-Condition in which a single great vessel leaves the heart and supplies the systemic and pulmonary circulation.
-Usually diagnosed prenatally via ultrasound.
-Neonates without prenatal diagnosis may present with aysmptomatic cyanosis, meaning they may be blue with saturations in the 75% to 85% range without respiratory distress

70
Q

Truncus Arteriosus

A

-Murmur may or may not be present depending on the origins and caliber of pulmonary arteries.
-Ausculation: first heart sound is normal and second is loud and single.
-Ausculation of lung fields may demonstrate rales.

71
Q

Goals of surgical interventions for truncus arteriosus

A

Neonatal & Pediatrics (4 decks)

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