AMA Category 1 Credit for Physicians Only.
ASRT CME is not available.
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The TEE showed:
LVEF 45% with global HK
Inferoseptal and Anteroseptal HK, Apical HK
LVID 5.3 cm
Posteriorly Eccentric MR Jet
Tethered,Restricted PMVL Movement, Especially at P2
The MR was severe.
AR was a trace
TR was a trace
This case is interesting because she has two issues: a DCM and severe MR. The cause of her respiratory failure is the severe MR. The MR is interesting in that MR that is from a DCM, the MR is due to the DCM where the papillary muscles pull down the anterior and posterior leaflet resulting in a central jet that is not eccentric in all views. She had more HK in the septal area which may explain her MR jet eccentricity. If one of the papillary muscles is more displaced than the other papillary muscle then the jet will be anteriorly or posteriorly directed, although it should not be eccentric. This jet has some posterior eccentricity as well as being posteriorly and centrally directed which makes on think the anterolateral papillary muscle is more affected. The PMVL is clearly restricted although it still maintains some motion. The AMVL is seen to be contacting the PMVL above the leaflet edge of the PMVL. The AMVL is restricted also, but to a lesser degree than the PMVL.
DCM is a chronic disease that can cause heart failure, arrhythmia, or sudden death. DCM has symptoms of SOB, edema, and fatigue. Physical exam will find signs of heart failure (JVD, S3, Rales, Hepatomegaly, Edema) Diagnosis is by physical exam, ECG, and echocardiography. Treatment is ACE-I, Beta-Blockers, Diuretics, and Digoxin, Pacemaker/Bi-ventricular pacing, and/or Defibrillator. Causes of DCM are CAD, infection, alcohol, valvular abnormalities, or idiopathic. Idiopathic DCM is about 1/3 of all DCM and may follow a familial pattern. Familial DCM may include rhythm abnormalities where syncope, sudden death may have occurred in the family. Familial DCM is usually autosomal dominant, with varying penetrance, involving men and women and multiple generations.
With pregnancy, the SVR drops, the HR, CO, Filling volumes increase. The CO increases 50%, the HR increases 20%, SVR drops as the MAP slightly increases. Filling pressures do not increase. All chambers of the heart increase in size (about 10% in LA, LV size, 20% increase in RA, RV size) The PV, MV and TV annulus increase in size. As the annulus diameters increase, regurgitation of the valves may increase. After delivery, all of the above changes revert towards normal in the postpartum period.
Peripartum cardiomyopathy is a DCM associated with pregnancy. PPCM must occur during the last trimester of pregnancy or in the postpartum period. If the CM occurs in the first two trimesters, it is not due to pregnancy and is usually due to other causes of heart disease (valvular, myocarditis, etc).
The symptoms of PPCM are SOB, edema, fatigue, nocturia, and palpitations. Many of the causes of PPCM is myocarditis which is confirmed with a heart biopsy. Risk factors include obesity, history of myocarditis, alcoholism, African American, malnourished. Other risk factors are > 30 years old, multiparous, multiple gestations, and PIH. Treatment depends on the severity and course of the PPCM. Some will improve(50%), others remain stable, while others will worsen. Treatment included IABP, Immunosuppressive therapy, heart transplant, LVADs. Medications include beta-blockers, diuretics, digoxin. Low salt diet, no alcohol and no smoking are also recommended. Subsequent pregnancies may result in return or worsening of the PPCM.
Patiens with heart failure from DCM or PPCM can undergo a pregnancy or repeat pregnancy as long as the LV function is reasonable and the response to treatment or cessation of pregnancy is favorable. While pregancy in a patient with CM is undesirable, it is not absolutely contraindicated.
Given the strong family history, it is most likely that this is a case of familial DCM rather than PPCM. However, PPCM cannot be ruled out. After her pregnancy, her MR became worse, either from the pregnancy induced changes in her mitral valve (unlikely since it does not seem to be due to mitral valvular annular dilation) or from her DCM becoming worse.
Since the valve could be repaired and will avoid a prosthetic valve until her DCM worsens, she underwent a minimally invasive MVR with a 30 mm rigid ring which resulted in a trace of MR after the mitral valve repair.
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Familial Dilated Cardiomyopathy
Familial dilated cardiomyopathy - Orphanet
Familial dilated cardiomyopathy: evidence for genetic and phenotypic heterogeneity. Heart Muscle Disease Study Group.
The Frequency of Familial Dilated Cardiomyopathy in a Series of Patients with Idiopathic Dilated Cardiomyopathy
Frequency and phenotypes of familial dilated cardiomyopathy.
Peripartum Cardiomyopathy - Circulation
Physiological changes in pregnancy