10. Heart Failure, Pulmonary HTN, Transplant Flashcards
(111 cards)
1
Q
Name 3 contraindications to heart transplant
A
- Elevated PVR
- Drug/alcohol addiction
- Active PE (resolved PE is NOT)
2
Q
In a Fontan patient, is PLE or plastic bronchitis a contraindication to tranplant?
A
No, but they do increase risk
3
Q
Most common indication for heart transplant in teenage years?
A
Cardiomyopathy
4
Q
Most common indication for heart transplant in patients >1?
A
Cardiomyopathy
-Dilated, restrictive, hypertrophic, non-compaction
5
Q
Most common indication for heart transplant in infants?
A
CHD (need has been decreasing)
6
Q
Name some findings of transplant rejection
A
- Tachycardia
- New murmurs (MR/TR)
- Evidence of congestion (hepatomegaly, JVD, abnormal CXR)
- Gallop rhythm
7
Q
What can cause a soft flow murmur in a patient early after heart transplant?
A
Anemia
8
Q
Most common echo findings in active transplant rejection?
A
- New effusion
- Increased wall thickness and ventricular mass
- Increased MR/TR
- Decreases in MV TDI velocities
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
10
Q
HLHS patient s/p transplant, right arm HTN and abdominal aortic doppler with diastolic flow continuation… cause?
A
Re-coarctation
11
Q
Contraindications to combined heart/lung transplant for pediatric patients
A
- Active TB
- Active malignancies
- Sepsis
- Severe systemic or neuromuscular diseases
- Multiorgan dysfunction
- Social concerns
*On ECMO at time of listing is controversial
12
Q
Name 3 common indications for a heart-lung transplant
A
- CF
- Pulm HTN
- Eisenmenger
13
Q
Transplant patient with irritability, tremulousness and seizures… which medication should you get a level to check for toxicity?
A
Tacrolimus
14
Q
True or False: B-blockers have little effect on tacrolimus/cyclosporine levels?
A
True
15
Q
What medications can increase tacrolimus/cyclosporine levels?
A
- Antifungals (fluconazole)
- Amiodarone
- Macrolides
- CCB
- Metoclopramide
16
Q
What medications can decrease tacrolimus/cyclosporine levels?
A
- Octreotide
- Some anticonvulsants (phenytoin, phenobarbital, carbamazepine)
- Antibiotics (nafcillin, IV bactrim)
17
Q
What is chronic inflammation of the bronchioles that results in fibrous deposition and ultimately obstructs airways?
A
Bronchiolitis obliterans
18
Q
What is a form of chronic rejection in lung transplant recipients?
A
Bronchiolitis obliterans
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
20
Q
Elevated exhaled NO levels and decreased mid-expiration flow rates (FEF 25 to 75) in a lung transplant patient?
A
Bronchiolitis obliterans
21
Q
Poor prognostic indications in restrictive cardiomyopathy?
A
- Significant cardiomegaly
- Pulmonary venous congestion on CXR
- Elevated LVEDP
- Degree of LA dilation
22
Q
What happens to TDI parameters in patients with restrictive cardiomyopathy?
A
Low
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
24
Q
What is the gold standard test to rule out rejection in transplant patient?
A
Myocardial biopsy
25
Besides myocardial biopsy, what other testing can suggest rejection of heart transplant?
1. Cardiac MRI
2. ECG (low-voltage QRS signals)
3. BNP (trend)
4. Echo
26
What 2 things should you consider in a transplant patient with a new-onset arrhythmia?
1. Rejection
2. Coronary artery vasculopathy
*Further out from transplant, likely coronary artery vasculopathy
27
Side effects of tacrolimus when levels too high?
1. Irritability
2. Tremulousness
3. Seizures
28
Side effects of azathioprine and mycophenolate?
1. Leukopenia
| 2. GI: Constipation, diarrhea, nausea
29
Side effects of sirolimus?
1. Diarrhea
| 2. Mouth sores
30
Side effects of prednisone?
1. Mood changes
2. Increased appetite
3. Increased blood glucose
4. Weight gain
5. Cushingoid appearance
6. Osteoporosis
31
Familial pulmonary HTN gene?
BMPR2
32
What chromosome is BMPR2 found on?
2
33
How is the BMPR2 gene (pulm HTN) inherited?
AD (low penetrance, 20%)
34
TBX5
Holt-Oram (large ASD + radial anomalies)
35
MLL2
Kabuki
36
JAG1
Alagille
37
PTNP11
Noonan
38
MOA Sildenafil?
PGE-5 inhibitor-
Blocks phosphodiesterase from degrading cyclic GMP in smooth muscle cells and promotes vasodilation
39
Name 3 endothelin receptor antagonists
1. Bosentan
2. Abrisentan
3. Macitentan
40
MOA ambrisentan?
Selective endothelin A receptor antagonist
41
MOA bosentan and macitentan?
Endothelin A and B receptor antagonist
42
What drug is used IV to restore balance of endogenous thromboxanes and prostacyclins to induce vasodilation?
Prostacyclin (prostaglanin I2)
43
What drug suppresses the production of prostaglandins and thromboxanes by irreversibly inactivating the cyclooxygenase enzyme
ASA
44
What is definitive therapy for restrictive cardiomyopathy?
Transplant
45
True or False: Without transplant, patients with restrictive cardiomyopathy have up to 50% mortality within 2-3 years of diagnosis of restrictive cardiomyopathy?
True
46
Besides transplant, what is the only medical therapy to help with symptoms in restrictive cardiomyopathy?
Limited diuresis
*Have to be cautious, patients are preload sensitive
47
Are ACEi helpful in restrictive cardiomyopathy?
No
48
Why is a liver US done as part of transplant evaluation?
Risk of long-term hepatic congestion
49
What should always be ruled out in patients with an elevated RVSP?
Pulmonary vein stenosis
*LAE points to ventricles/cardiomyopathy as issue versus pulmonary veins however
50
Dilated cardiomyopathy, on enalapril, carvedilol, spironolactone and lasix... what medication can be added if continued HF symptoms?
Digoxin
51
Should you start digoxin in a HF patient who is asymptomatic?
No
52
Which HF patients may need lower doses of digoxin?
1. Renal dysfunction
2. Taking carvedilol
3. Taking amiodarone
53
In HF patients, when would you wean medications?
Only if ventricular function has improved
54
When can milrinone be used in HF patients in the outpatient setting?
Bridge to transplant
55
MOA spironolactone?
Inhibits aldosterone at distal tubule
*K-sparing, mild diuretic effect (used concurrently with loop or thiazide)
56
Where does acetazolamide act?
Proximal convolute tubule (carbonic anhydrase inhibitor)
57
Where do thiazide diuretics act?
Distal convoluted tubule (inhibit Na/Cl transport)
58
Where do loop diuretics act?
Thick ascending limb of loop of Henle (inhibit Cl-Na-K cotransport)
59
Where to Ca channel blockers act?
L-type Ca channels in heart
60
What agents can cause methemoglobinemia?
1. Topical anesthetic agnents- Lidocaine, benzocaine, prilocaine
2. Dapsone
61
When is dapsone used?
PCP prophylaxis in patients who can't use Bactrim
62
How do you treat methmeoglobinemia?
1. Stop offending agent
| 2. Methylene blue
63
How does methemoglobinemia present?
Acute hypoxia
64
MOA Enalapril?
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
What do you need to counsel females about who take ACEi?
Avoid pregnancy
66
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?
Grade 1R (mild)
67
What grade of rejection is defined by biopsy with 2 or more foci of infiltrate with associated myocyte damage following heart transplant?
Grade 2R (moderate)
68
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?
Grade 3R (severe)
69
Above what rejection grade in heart transplant should be treated?
2R or greater
70
How is heart transplant rejection initially treated?
Pulsed steroids
71
True or False: Grade 1R rejection after heart transplant shouldn't be treated
False- Decision is controversial and depends on several factors like prior biopsies, protocols at the institution and comorbidities
72
If familial/inherited, what is the most common pattern of inheritance in DCM?
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
What is the most common complication of azathioprine and mycophenolate?
Leukopenia
74
Besides leukopenia, what is another common side effect of azathioprine and mycophenolate?
GI: Constipation, diarrhea, nausea
75
What rejection medication can cause irritability and tremulousness if levels are too high?
Tacrolimus
76
List side effects of prednisone
0Mood changes
- Increased appetite
- Increased blood glucose
- Weight gain
- Cushingoid appearance
- Long term use associated with osteoporosis
77
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)?
Minimize immunosuppression therapy
78
True or False: Post-transplant lymphoproliferative disorder is a significant cause of graft loss and death after transplant?
True
79
What has been shown to improve survival in the settling of PTLD following transplant?
Reduction in immunosuppression early after transplant
80
The majority of lymphomas after transplant are related to what?
EBV
81
Can someone with PTLD be listed for re-transplant?
Controversial (institution-dependent)
82
How is Barth syndrome inherited?
X-linked recessive
83
DCM or LVNC, hypotonia, proximal muscle weakess, neutropenia, short stature?
Barth
*Age and severity vary widely
84
What gene mutation is seen with Barth syndrome?
TAZ mutation
*Encodes Tafazzin protein
85
In a patient with heart failure and a dry hacking cough, what medication adjustment should be made?
Switch from ACEi to ARB
86
Why does ACEi cause cough?
- 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
Cough due to ACEi usually improves in what timeframe?
Within a week
88
True or False: ACEi induced cough has a high rate of recurrence
True- Up to 67% if second challenge of medication given
89
Management options for coronary artery vasculopathy following transplant?
1. ASA
2. Statin drug (pravastatin)
3. Switching from a calcineurin inhibitor (cyclosporine, tacrolimus, pimecrolimus) to an mTOR inhibitor (sirolimus or everolimus)
90
Coronary angiography with areas of diffuse coronary luminal narrowing in multiple branches and biopsy with no evidence rejection after transplant?
Severe coronary artery vasculopathy
91
Can you do stenting for coronary artery vasculopathy following transplant?
In certain situations... typically doesn't have long-term benefit due to high instance of re-stenosis
92
What is the best option for severe coronary artery vasculopathy following transplant?
List for re-transplant
93
What are 2 treatment options for rejection after transplant?
1. Steroids
| 2. Plasmapheresis
94
True or False: Many patients with coronary artery vasculopathy following transplant get presumptive treatment for rejection
True (with hope for clinical improvement)
*This shouldn't be done in lieu of listing for re-transplant
95
What should you consider in a post-transplant patient with irritability and prominent venous distention in head/neck?
SVC obstruction
*Can be seen with SVC thrombus or obstruction at SVC anastomosis
96
Risk for SVC obstruction following transplant increases in what 2 patients groups?
1. Prior SVC intervention (Glenn)
| 2. Bicaval anastomosis for transplant
97
What % of pediatric heart transplant recipients require further intervention on SVC?
10%
98
What is 1st line treatment for SVC obstruction following heart transplant?
Angioplasty +/- stenting
99
What drug inhibits the Cl-Na-K cotransport in the thick ascending limb of the loop of Henle?
Loop diuretic (Lasix)
100
What is the preferred primary diuretic therapy in systolic heart failure?
Loops
101
What drugs inhibit Na/Cl transport in the distal convoluted tubule?
Thiazides
102
Where do carbonic anhydrase inhibitors (like acetazolamide) act?
In the proximal convoluted tubule
103
How do K sparing diuretics like spironolactone work?
Inhibit aldosterone at distal tubule
* Reduce K loss in urine
* Mild diuretic effect... usually used with loop or thiazide
104
What do CCB do?
Block l-type Ca channels in the heart
105
MOA for carvedilol?
Non-selective B blockade
| Alpha blockade
106
MOA of metoprolol?
Selective blockade of B1
| Little to no effect on B2
107
MOA ACEi?
Inhibition of ACE
| Prevents conversion of angiotensin I to angiotensin II
108
What drug has direct vasodilation of arterioles with subsequent decrease in systemic resistance?
Hydralazine
109
What does hydralazine do to afterload, CO, filling pressure?
Decreases afterload
Increases CO
Decrease filling pressures
*Dilates peripheral arteries
110
What drug blocks epithelial Na channels in late distal convoluted tubule and collecting duct?
Amiloride
111
What is the effect of amiloride?
- 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)
