Term
| What was Eisenmenger's first name? |
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Definition
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Term
| In developed countries, how does congenital heart disease rank in terms of frequency as a cause of PAH? |
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Definition
| Next to collagen vascular disease (chiefly systemic sclerosis), CHD is the most common cause of PAH. |
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Term
| Which of the following is most likely to result in PAH? Perimembranous VSD, sinus venous ASD, complete atrioventricular canal defect. |
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Definition
| AV canal defects have the highest incidence of PAH followed by VSDs and ASDs. In general, PAH is more likely to develop with post-tricuspid defects, complex defects, and, of course, large defects. Aortopulmonary window and truncus arteriosus have near universal PAH. |
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Term
True or False:
Closure of a large VSD in the first few months of life precludes the development of PAH. |
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Definition
False
For some reason, PAH may fail to reverse or even progress after correction of a shunt. Fortunately, that is rare. |
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Term
| What are the 4 groups of CHD with PAH according to current guidelines? |
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Definition
1. Eisenmenger syndrom3
2. PAH and coexisting shunt
3. PAH and small shunt (VSD <1 cm D, ASD <2 cm D)
4. PAH after corrective surgery |
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Term
| When PAH recurs or develops after corrective surgery is the prognosis better or worse than that of pts with PAH and uncorrected lesions. |
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Definition
Worse.
That is one reason why much thought is given to closing shunts when PAH is present |
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Term
| Why are post-tricuspid shunts more likely to cause PAH then pre-tricuspid shunts? |
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Definition
| Pre-tricuspid shunts (ASDs, anomalous PV return) cause volume overload of the PA, post-tricuspid shunts cause volume and pressure overload. |
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Term
| In Eisenmenger's syndrome, are PA pressure and resistance lower than, the same as, or higher than systemic pressure and resistance? |
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Definition
| The same as. When parity is reached, a bidirectional shunt occurs. |
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Term
| How can a bidirectional shunt benefit a pt with Eisenmenger's? |
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Definition
| Right to left shunts increase systemic stroke volume and reduce the load on the RV. These benefits are purchased at the cost of cyanosis and the complications cyanosis brings. |
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Term
| How does the prognosis in Eisenmenger's syndrome compare with that of idiopathic PAH? |
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Definition
| Pts with Eisenmenger's syndrome have a better prognosis than pts with idiopathic PAH. Possibly, that is because when PAH develops in childhood, the RV makes a more successful adaptation to high pressures. |
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Term
| What are the hematologic consequences of hypoxemia and cyanosis? |
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Definition
erythrocytosis
clotting disorders
hyperviscosity |
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Term
| What are the neurologic complications of Eisenmenger's syndrome |
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Definition
headache from hyper viscosity
cerebral abscess
stroke |
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Term
True or False
Endocarditis is rare in Eisenmenger's syndrome |
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Definition
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Term
| A pt with Eisenmenger's syndrome has aching in the thigh. What problem associated with Eisenmenger's syndrome could explain that? |
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Definition
| hypertrophic osteoarthropathy. |
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Term
| Erythrocytosis is associated with which rheumatologic condition? |
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Definition
| Gout. Increased RBC production and decreased urate clearance in pts with Eisenmenger's syndrome leads to elevated uric acid levels. |
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Term
True or False
Because of the high risk of PA thrombosis, pts with Eisenmenger's syndrome should be anti coagulated. |
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Definition
| Pts with Eisenmenger's syndrome are liable to both clotting and hemoptysis, so anticoagulation is not a routine recommendation. Thrombosis is most likely in an older person with RV failure and very dilated PAs. When thrombosis is found in that setting, anticoagulation may be used if hemoptysis is absent or minimal. |
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Term
True or False
Nocturnal home 02 has been shown to improve sx and longevity in Eisenmenger's syndrome |
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Definition
False.
Nocturnal home 02 does not affect mortality.
Though a right-left shunt is present, hypoxemia may improve with 02. When that has been documented and when sx improve, home 02 may be prescribed. |
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Term
| A pt has a HCT of 68% and complains of headache and confusion. Should you proceed with phlebotomy? |
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Definition
| Maybe, but only after excluding Fe deficiency and volume depletion. HA and confusion are compatible with hyper viscosity, but hyper viscosity is more often due to Fe deficiency or volume depletion than to erythrocytosis. When phlebotomy is performed, the volume removed is replaced with isotonic saline. |
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Term
| Name two causes of hemoptysis in pts with Eisenmenger's syndrome |
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Definition
1. In situ thrombosis of a pulmonary artery with infarction and
2. Aortopulmonary collaterals. These can be treated with embolization |
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Term
| What is the drug with the strongest evidence for benefit in Eisenmenger's syndrome |
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Definition
| Bosentan. It is the only one with a RCT. Sildenafil has been studied in open label cohort studies and there are case series for epoprostenol. Combination therapy has been tried and appears reasonable if hit or miss. |
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Term
| A 35 yr old woman with mild doe presents with an ASD. Her PA pressure is 60/25, PVR 4.7 WU, and Qp:Os 1.5. Should she undergo closure of the ASD? |
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Definition
| Yes. Current European guidelines recommend closure of an ASD or VSD when the left to right shunt is at least 1.5 and the WU are <5. |
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Term
| A man with a VSD and severe sob presents with a PA pressure of 70/30, PVR of 6 WU, and Qp:Qs of 1.5. His brachial artery pressure is 120/80. Should he undergo closure of the VSD? |
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Definition
| Who knows. The Europeans would give him a IIB recommendation for closure. Here the threshold value is the ratio of pulmonary to systemic systolic pressure which is < 2/3. The same ratio is applied to pulmonary/systemic resistance. |
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Term
| Another pt with a VSD presents with similar hemodynamics, but now the systemic pressure is 100 and SVR is 8.8. What further testing or treatment can be considered? |
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Definition
| In the cath lab, pulmonary dilators may be given such as inhaled nitric oxide. Alternatively, the pt may be placed on bosentan with or without sildenfafil and restudied in a few months. If the pulmonary/systemic pressure and resistance ratios fall below 2/3, closure of the VSD may be a good idea (or not) |
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