Eisenmenger's syndrome
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Introduction
A congenital anomaly consisting of left to right cardiac shunt, pulmonary hypertension, & right ventricular hypertrophy.
Etiology
- patent ductus arteriosus
- ventricular septal defect
- atrial septal defect (pulmonary hypertension infrequent)[3]
Pathology
- large left to right cardiac shunt producing pulmonary hypertension & irreversible pulmonary vascular disease
- pulmonary hypertension causes left to right shunt to reverse so that blood flows from right to left, resulting in cyanotic heart disease
- compensatory erythrocytosis develops
- death commonly occurs in 3rd or 4th decade of life
Clinical manifestations
- symptoms develop during the 1st few years of life
- cyanosis of lower extremities
- clubbing of toes
- dyspnea
- exercise induced syncope
- arrhythmia
- systolic murmur heard best at 4th left intercostal space
- initially loud, but diminishes in intensity when shunt reverses from left->right to right->left
- accentuated P2 heart sound
- ejection click
- accentuated precordial thrust of right ventricle
Laboratory
- complete blood count
- erythrocytosis
- normal blood hemoglobin in patients with cyanotic heart disease is 18-20 g/dL
- normal hematocrit is 60-65%
- iron studies if relative anemia
- iron deficiency if repetitively phlebomotomized
- menorrhagia can also result in iron deficiency
Diagnostic procedures
Radiology
- chest X-ray
- enlarged pulmonary arteries
- decreased pulmonary vascularity
- right ventricular enlargement
- echocardiography
Complications
Management
- phlebotomy
- avoid phlebotomy if possible
- leads to iron deficiency
- iron-deficient erythrocytes are more rigid increasing risk of stroke
- phlebotomy may be necessary in patients with hemoglobin > 20 g/dL & hematocrit > 65% with symptoms of hyperviscosity)
- correct volume depletion prior to decision for phlebotomy
- replace fluid concomittantly with phlebomtomy
- hypotension or syncope may be fatal
- treat iron deficiency with short course of iron until serum ferritin & transferrin saturation normalize[3][5]
- no proven benefit to increasing supplemental oxygen in patients with cyanotic heart disease[8]
- pulmonary vasodilation therapy (see pulmonary hypertension)
- surgery
- large VSD with left to right shunt & pulmonary hypertension should not be surgically closed
- clinical deterioration will result
- heart or heart-lung transplantation[3]
- patients are generally not surgical candidates
- large VSD with left to right shunt & pulmonary hypertension should not be surgically closed
- avoid pregnancy:
- peripartum maternal mortality is high
- right of maternal mortality is 30-50%[3]
- intravenous access
- use air filters to prevent paradoxical air embolism*[3]
- special considerations for air travel
- pressurized cabin
- consider supplemental oxygen
* patients with cardiac right to left shunt at risk for paradoxical air embolism[3]
More general terms
Additional terms
References
- ↑ DeGowin & DeGowin's Diagnostic Examination, 6th edition, RL DeGowin (ed), McGraw Hill, NY 1994, pg 388. 881
- ↑ Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 46-47
- ↑ 3.0 3.1 3.2 3.3 3.4 3.5 3.6 Medical Knowledge Self Assessment Program (MKSAP) 11, 15, 16, 17, 18. American College of Physicians, Philadelphia 1998, 2009, 2012, 2015, 2018.
- ↑ Dimopoulos K, Inuzuka R, Goletto S et al Improved survival among patients with Eisenmenger syndrome receiving advanced therapy for pulmonary arterial hypertension. Circulation. 2010 Jan 5;121(1):20-5 PMID: https://www.ncbi.nlm.nih.gov/pubmed/20026774
- ↑ 5.0 5.1 Tay EL, Peset A, Papaphylactou M et al Replacement therapy for iron deficiency improves exercise capacity and quality of life in patients with cyanotic congenital heart disease and/or the Eisenmenger syndrome. Int J Cardiol. 2011 Sep 15;151(3):307-12 PMID: https://www.ncbi.nlm.nih.gov/pubmed/20580108
- ↑ Srinivas SK, Manjunath CN. Differential clubbing and cyanosis: classic signs of patent ductus arteriosus with Eisenmenger syndrome. Mayo Clin Proc. 2013 Sep;88(9):e105-6. PMID: https://www.ncbi.nlm.nih.gov/pubmed/24001503
- ↑ D'Alto M1, Romeo E, Argiento P et al Bosentan-sildenafil association in patients with congenital heart disease-related pulmonary arterial hypertension and Eisenmenger physiology. Int J Cardiol. 2012 Mar 22;155(3):378-82 PMID: https://www.ncbi.nlm.nih.gov/pubmed/21081251
- ↑ 8.0 8.1 NEJM Knowledge+