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Peds Cardiology > 10. Heart Failure, Pulmonary HTN, Transplant > Flashcards

10. Heart Failure, Pulmonary HTN, Transplant Flashcards

1
Q

Name 3 contraindications to heart transplant

A
  1. Elevated PVR
  2. Drug/alcohol addiction
  3. Active PE (resolved PE is NOT)
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2
Q

In a Fontan patient, is PLE or plastic bronchitis a contraindication to tranplant?

A

No, but they do increase risk

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3
Q

Most common indication for heart transplant in teenage years?

A

Cardiomyopathy

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4
Q

Most common indication for heart transplant in patients >1?

A

Cardiomyopathy

-Dilated, restrictive, hypertrophic, non-compaction

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5
Q

Most common indication for heart transplant in infants?

A

CHD (need has been decreasing)

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6
Q

Name some findings of transplant rejection

A
  1. Tachycardia
  2. New murmurs (MR/TR)
  3. Evidence of congestion (hepatomegaly, JVD, abnormal CXR)
  4. Gallop rhythm
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7
Q

What can cause a soft flow murmur in a patient early after heart transplant?

A

Anemia

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8
Q

Most common echo findings in active transplant rejection?

A
  1. New effusion
  2. Increased wall thickness and ventricular mass
  3. Increased MR/TR
  4. Decreases in MV TDI velocities
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9
Q

What is a potential complication in patients with HLHS after transplant?

A

Recoarctation of the aorta- may need cath for balloon angioplasty or stenting

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10
Q

HLHS patient s/p transplant, right arm HTN and abdominal aortic doppler with diastolic flow continuation… cause?

A

Re-coarctation

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11
Q

Contraindications to combined heart/lung transplant for pediatric patients

A
  1. Active TB
  2. Active malignancies
  3. Sepsis
  4. Severe systemic or neuromuscular diseases
  5. Multiorgan dysfunction
  6. Social concerns

*On ECMO at time of listing is controversial

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12
Q

Name 3 common indications for a heart-lung transplant

A
  1. CF
  2. Pulm HTN
  3. Eisenmenger
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13
Q

Transplant patient with irritability, tremulousness and seizures… which medication should you get a level to check for toxicity?

A

Tacrolimus

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14
Q

True or False: B-blockers have little effect on tacrolimus/cyclosporine levels?

A

True

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15
Q

What medications can increase tacrolimus/cyclosporine levels?

A
  1. Antifungals (fluconazole)
  2. Amiodarone
  3. Macrolides
  4. CCB
  5. Metoclopramide
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16
Q

What medications can decrease tacrolimus/cyclosporine levels?

A
  1. Octreotide
  2. Some anticonvulsants (phenytoin, phenobarbital, carbamazepine)
  3. Antibiotics (nafcillin, IV bactrim)
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17
Q

What is chronic inflammation of the bronchioles that results in fibrous deposition and ultimately obstructs airways?

A

Bronchiolitis obliterans

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18
Q

What is a form of chronic rejection in lung transplant recipients?

A

Bronchiolitis obliterans

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19
Q

What is the clinical presentation of bronchiolitis obliterans?

A

Non-specific… can be URI like at first, increase in exertional dyspnea, decreased daily spirometry values

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20
Q

Elevated exhaled NO levels and decreased mid-expiration flow rates (FEF 25 to 75) in a lung transplant patient?

A

Bronchiolitis obliterans

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21
Q

Poor prognostic indications in restrictive cardiomyopathy?

A
  1. Significant cardiomegaly
  2. Pulmonary venous congestion on CXR
  3. Elevated LVEDP
  4. Degree of LA dilation
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22
Q

What happens to TDI parameters in patients with restrictive cardiomyopathy?

A

Low

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23
Q

True or false, RAE on echo and TR murmur are poor prognostic indicators in restrictive cardiomyopathy?

A

False, but can be indicative of degree of RV dysfunction

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24
Q

What is the gold standard test to rule out rejection in transplant patient?

A

Myocardial biopsy

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25
Q

Besides myocardial biopsy, what other testing can suggest rejection of heart transplant?

A
  1. Cardiac MRI
  2. ECG (low-voltage QRS signals)
  3. BNP (trend)
  4. Echo
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26
Q

What 2 things should you consider in a transplant patient with a new-onset arrhythmia?

A
  1. Rejection
  2. Coronary artery vasculopathy

*Further out from transplant, likely coronary artery vasculopathy

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27
Q

Side effects of tacrolimus when levels too high?

A
  1. Irritability
  2. Tremulousness
  3. Seizures
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28
Q

Side effects of azathioprine and mycophenolate?

A
  1. Leukopenia

2. GI: Constipation, diarrhea, nausea

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29
Q

Side effects of sirolimus?

A
  1. Diarrhea

2. Mouth sores

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30
Q

Side effects of prednisone?

A
  1. Mood changes
  2. Increased appetite
  3. Increased blood glucose
  4. Weight gain
  5. Cushingoid appearance
  6. Osteoporosis
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31
Q

Familial pulmonary HTN gene?

A

BMPR2

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32
Q

What chromosome is BMPR2 found on?

A

2

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33
Q

How is the BMPR2 gene (pulm HTN) inherited?

A

AD (low penetrance, 20%)

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34
Q

TBX5

A

Holt-Oram (large ASD + radial anomalies)

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35
Q

MLL2

A

Kabuki

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36
Q

JAG1

A

Alagille

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37
Q

PTNP11

A

Noonan

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38
Q

MOA Sildenafil?

A

PGE-5 inhibitor-

Blocks phosphodiesterase from degrading cyclic GMP in smooth muscle cells and promotes vasodilation

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39
Q

Name 3 endothelin receptor antagonists

A
  1. Bosentan
  2. Abrisentan
  3. Macitentan
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40
Q

MOA ambrisentan?

A

Selective endothelin A receptor antagonist

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41
Q

MOA bosentan and macitentan?

A

Endothelin A and B receptor antagonist

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42
Q

What drug is used IV to restore balance of endogenous thromboxanes and prostacyclins to induce vasodilation?

A

Prostacyclin (prostaglanin I2)

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43
Q

What drug suppresses the production of prostaglandins and thromboxanes by irreversibly inactivating the cyclooxygenase enzyme

A

ASA

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44
Q

What is definitive therapy for restrictive cardiomyopathy?

A

Transplant

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45
Q

True or False: Without transplant, patients with restrictive cardiomyopathy have up to 50% mortality within 2-3 years of diagnosis of restrictive cardiomyopathy?

A

True

46
Q

Besides transplant, what is the only medical therapy to help with symptoms in restrictive cardiomyopathy?

A

Limited diuresis

*Have to be cautious, patients are preload sensitive

47
Q

Are ACEi helpful in restrictive cardiomyopathy?

A

No

48
Q

Why is a liver US done as part of transplant evaluation?

A

Risk of long-term hepatic congestion

49
Q

What should always be ruled out in patients with an elevated RVSP?

A

Pulmonary vein stenosis

*LAE points to ventricles/cardiomyopathy as issue versus pulmonary veins however

50
Q

Dilated cardiomyopathy, on enalapril, carvedilol, spironolactone and lasix… what medication can be added if continued HF symptoms?

A

Digoxin

51
Q

Should you start digoxin in a HF patient who is asymptomatic?

A

No

52
Q

Which HF patients may need lower doses of digoxin?

A
  1. Renal dysfunction
  2. Taking carvedilol
  3. Taking amiodarone
53
Q

In HF patients, when would you wean medications?

A

Only if ventricular function has improved

54
Q

When can milrinone be used in HF patients in the outpatient setting?

A

Bridge to transplant

55
Q

MOA spironolactone?

A

Inhibits aldosterone at distal tubule

*K-sparing, mild diuretic effect (used concurrently with loop or thiazide)

56
Q

Where does acetazolamide act?

A

Proximal convolute tubule (carbonic anhydrase inhibitor)

57
Q

Where do thiazide diuretics act?

A

Distal convoluted tubule (inhibit Na/Cl transport)

58
Q

Where do loop diuretics act?

A

Thick ascending limb of loop of Henle (inhibit Cl-Na-K cotransport)

59
Q

Where to Ca channel blockers act?

A

L-type Ca channels in heart

60
Q

What agents can cause methemoglobinemia?

A
  1. Topical anesthetic agnents- Lidocaine, benzocaine, prilocaine
  2. Dapsone
61
Q

When is dapsone used?

A

PCP prophylaxis in patients who can’t use Bactrim

62
Q

How do you treat methmeoglobinemia?

A
  1. Stop offending agent

2. Methylene blue

63
Q

How does methemoglobinemia present?

A

Acute hypoxia

64
Q

MOA Enalapril?

A

ACEi- Decreases the amount of angiotensin I that is converted to angiotensin II

  • Angiotensin II is a vasoconstrictor, ACEi promote vasodilation
  • ACEi decrease degradation of bradykinins
  • ACEi reduce production of aldosterone
65
Q

What do you need to counsel females about who take ACEi?

A

Avoid pregnancy

66
Q

What grade of rejection is defined by biopsy with intersitial and/or perivascular infiltrates with up to one focus of myocyte damage following heart transplant?

A

Grade 1R (mild)

67
Q

What grade of rejection is defined by biopsy with 2 or more foci of infiltrate with associated myocyte damage following heart transplant?

A

Grade 2R (moderate)

68
Q

What grade of rejection is defined by biopsy with diffuse infiltrate with multifocal myocyte damage (with or without edema, hemorrhage or vasculitis) following heart transplant?

A

Grade 3R (severe)

69
Q

Above what rejection grade in heart transplant should be treated?

A

2R or greater

70
Q

How is heart transplant rejection initially treated?

A

Pulsed steroids

71
Q

True or False: Grade 1R rejection after heart transplant shouldn’t be treated

A

False- Decision is controversial and depends on several factors like prior biopsies, protocols at the institution and comorbidities

72
Q

If familial/inherited, what is the most common pattern of inheritance in DCM?

A

AD- 50% risk of developing DCM for children of affected individual

*There are rare forms inherited as an x-linked or autosomal recessive pattern (often associated with neuromuscular disease or metabolic derangements)

73
Q

What is the most common complication of azathioprine and mycophenolate?

A

Leukopenia

74
Q

Besides leukopenia, what is another common side effect of azathioprine and mycophenolate?

A

GI: Constipation, diarrhea, nausea

75
Q

What rejection medication can cause irritability and tremulousness if levels are too high?

A

Tacrolimus

76
Q

List side effects of prednisone

A

0Mood changes

  • Increased appetite
  • Increased blood glucose
  • Weight gain
  • Cushingoid appearance
  • Long term use associated with osteoporosis
77
Q

When there is EBV mismatch between donor and recipient in heart transplant, what is done to reduce the likelihood of development of post-transplant lymphoproliferative disorder (PTLD)?

A

Minimize immunosuppression therapy

78
Q

True or False: Post-transplant lymphoproliferative disorder is a significant cause of graft loss and death after transplant?

A

True

79
Q

What has been shown to improve survival in the settling of PTLD following transplant?

A

Reduction in immunosuppression early after transplant

80
Q

The majority of lymphomas after transplant are related to what?

A

EBV

81
Q

Can someone with PTLD be listed for re-transplant?

A

Controversial (institution-dependent)

82
Q

How is Barth syndrome inherited?

A

X-linked recessive

83
Q

DCM or LVNC, hypotonia, proximal muscle weakess, neutropenia, short stature?

A

Barth

*Age and severity vary widely

84
Q

What gene mutation is seen with Barth syndrome?

A

TAZ mutation

*Encodes Tafazzin protein

85
Q

In a patient with heart failure and a dry hacking cough, what medication adjustment should be made?

A

Switch from ACEi to ARB

86
Q

Why does ACEi cause cough?

A
  • Mechanism not fully determined, thought to be related to increased local concentration of kinins and substance P (may induced bronchial irritation)
  • May also be related to arachidonic acid pathway activation…lead to elevated levels of thromboxane and subsequent bronchoconstriction
87
Q

Cough due to ACEi usually improves in what timeframe?

A

Within a week

88
Q

True or False: ACEi induced cough has a high rate of recurrence

A

True- Up to 67% if second challenge of medication given

89
Q

Management options for coronary artery vasculopathy following transplant?

A
  1. ASA
  2. Statin drug (pravastatin)
  3. Switching from a calcineurin inhibitor (cyclosporine, tacrolimus, pimecrolimus) to an mTOR inhibitor (sirolimus or everolimus)
90
Q

Coronary angiography with areas of diffuse coronary luminal narrowing in multiple branches and biopsy with no evidence rejection after transplant?

A

Severe coronary artery vasculopathy

91
Q

Can you do stenting for coronary artery vasculopathy following transplant?

A

In certain situations… typically doesn’t have long-term benefit due to high instance of re-stenosis

92
Q

What is the best option for severe coronary artery vasculopathy following transplant?

A

List for re-transplant

93
Q

What are 2 treatment options for rejection after transplant?

A
  1. Steroids

2. Plasmapheresis

94
Q

True or False: Many patients with coronary artery vasculopathy following transplant get presumptive treatment for rejection

A

True (with hope for clinical improvement)

*This shouldn’t be done in lieu of listing for re-transplant

95
Q

What should you consider in a post-transplant patient with irritability and prominent venous distention in head/neck?

A

SVC obstruction

*Can be seen with SVC thrombus or obstruction at SVC anastomosis

96
Q

Risk for SVC obstruction following transplant increases in what 2 patients groups?

A
  1. Prior SVC intervention (Glenn)

2. Bicaval anastomosis for transplant

97
Q

What % of pediatric heart transplant recipients require further intervention on SVC?

A

10%

98
Q

What is 1st line treatment for SVC obstruction following heart transplant?

A

Angioplasty +/- stenting

99
Q

What drug inhibits the Cl-Na-K cotransport in the thick ascending limb of the loop of Henle?

A

Loop diuretic (Lasix)

100
Q

What is the preferred primary diuretic therapy in systolic heart failure?

A

Loops

101
Q

What drugs inhibit Na/Cl transport in the distal convoluted tubule?

A

Thiazides

102
Q

Where do carbonic anhydrase inhibitors (like acetazolamide) act?

A

In the proximal convoluted tubule

103
Q

How do K sparing diuretics like spironolactone work?

A

Inhibit aldosterone at distal tubule

  • Reduce K loss in urine
  • Mild diuretic effect… usually used with loop or thiazide
104
Q

What do CCB do?

A

Block l-type Ca channels in the heart

105
Q

MOA for carvedilol?

A

Non-selective B blockade

Alpha blockade

106
Q

MOA of metoprolol?

A

Selective blockade of B1

Little to no effect on B2

107
Q

MOA ACEi?

A

Inhibition of ACE

Prevents conversion of angiotensin I to angiotensin II

108
Q

What drug has direct vasodilation of arterioles with subsequent decrease in systemic resistance?

A

Hydralazine

109
Q

What does hydralazine do to afterload, CO, filling pressure?

A

Decreases afterload
Increases CO
Decrease filling pressures

*Dilates peripheral arteries

110
Q

What drug blocks epithelial Na channels in late distal convoluted tubule and collecting duct?

A

Amiloride

111
Q

What is the effect of amiloride?

A
  • Inhibits Na reabsorption from lumen in late distal convoluted tubule and collecting duct
  • Reduces net negative potential of lumen of tubule (reduces both K and H excretion)

Peds Cardiology (20 decks)

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