Two Cardiology Zebras ERIC MARTIN MD

Transcription

1 Two Cardiology Zebras ERIC MARTIN MD

2 Disclosures Bayer Gilead Sciences NIH Vascular Dynamics, In. Employer Iowa Heart Center/Mercy Des Moines

3 Zebra # 1

4 History CC: 52-year-old man seen in consultation for a 3 year history of heart-failure FHx: Father had MI at 43 years of age died of "second heart attack" 6 months later. Paternal grandfather and one uncle both died in their 40s of "myocardial infarction. His son had patent ductus arteriosus.

5 Physical Examination Middle-aged WM in no acute distress Heart rate: 60 bpm with frequent ectopic beats BP: 120/80 mm Hg, pulse 96 Lungs clear Mild cardiomegally, no murmur but loud S4 gallop was present. No peripheral edema noted. No jugular-vein distention or hepato-jugular reflux.

6 Routine Studies ECG: normal sinus rhythm, 1 AV block, frequent PVCs in an intermittent bigeminal pattern, non-specific ST and T-wave changes Chest x-ray: minimally enlarged cardiac silhouette with clear lungs

7 ECHO Cardiogram

8 Contrast Enhanced ECHO Cardiogram

9 Differential Diagnoses Mural thrombi False Tendon Apical Hypertrophic Cardiomyopathy Cardiac Fibroma Cardiac Metastases Loeffler Endocarditis LV Noncompaction

10 Left Ventricular Non-Compaction (LVNC) Synonyms Noncompaction of the LV Myocardium Left Ventricular Hyper Trabeculation Spongy Myocardium

11 Left Ventricular Non-Compaction (LVNC) Incidence or prevalence is uncertain Estimates vary between 0.12 and 2.2/100,000 May be isolated or associated with other congenital cardiac and non-cardiac abnormalities Autosomal dominant form of transmission Multiple genetic defects have been documented

12 Proposed Etiology A congenital disorder of endomyocardial embryogenesis. The postulated but unproven cause: Arrest of the normal compaction of the loose interwoven mesh of myocardial fibers en utero during days 32 to 70 of fetal development. There is little direct evidence to support this proposition

13 Location of Lesions in 7 patients with LVNC The basal septum and basal inferior wall are spared Heart 2001;86:

14 A: Anastomosing broad trabeculae Multiple Phenotypes: Gross Appearance B :Coarse trabeculae resembling multiple papillary muscles Virmani R et al Hum Pathol Apr;36(4):403-11

15 LVNC: Gross Appearance C: interlacing smaller muscle bundles resembling a sponge D: The trabeculae can only be appreciated viewed en face Virmani R et al Hum Pathol Apr;36(4):403-11

16 Histopathology Note the thin compacted normal outer layer of myocardium and the endocardial (non-compacted) layer. There is scar tissue within the trabeculations (asterisks) and in the subendocardial area but not in the epicardial zone. Heart 2001;86:

17 Clinical Classification Isolated LVNC Typically presents in adulthood No communication between coronary arteries and LV chamber.

18 Clinical Classification Complex LVNC-Reported with various forms of congenital heart disease. Often seen in children PDA Bicuspid aortic valve disease Multiple type of complex congenital heart disease particularly with RV outflow tract problems Trabeculations may communicate with coronary arteries creating a coronary-cameral shunt (LV)

19 Clinical Imaging in LVNC Left heart cath contrast LV angiogram TTE and TTE with Doppler Cardiac Magnetic Resonance Imaging

20 Contrast Left Ventriculogram Left ventricular angiography RAO projection. Left ventricular angiography LAO projections.

21 Jenni, R et al Heart 86 (2001)666 Criteria for the Dx Isolated LVNC A 2 layer structure Compacted thin epicardial band and a much thicker noncompacted endocardial layer Deep endomyocardial spaces Maximal end-systolic ratio of noncompacted to compacted layers (>2:1 ratio by echo and 2.3:1 by CMR). CMR or color Doppler evidence of deeply perfused intertrabecular recesses.

22 Trans Esophageal Echocardiogram On transgastric two chamber view, the anteriomedial papillary muscle is poorly defined and characterized by the presence of numerous separated bands (arrows) inserting in to the anterior wall near the apex. The absence of a well defined papillary muscle is common in LVNC

23 Color Doppler in LVNC Color Doppler study shows typical flow away from the ventricular cavity into the deep spaces between the prominent trabeculation during diastole (in A represented by a red signal) with flow back into the ventricle during systole (in B, blue signal).

24 Trans Esophageal Echocardiogram Note the reduced left ventricular function and the appearance of left ventricular hypertrophy. The fingers of myocardium extend into the cavity approximately 2 to 3 cm.

25 Contrast Enhanced CMR in LVNC White Blood Technique

26 LVNC on CMR Black Blood Technique

27 CMR Cine 4 Chamber View

28 Detection of Thrombi in NCLV Heart 2005;91:e4

29 Detection of Thrombi in LVNC Heart 2005;91:e4

30 CMR with Gadolinium Delayed Enhancement A-Single frame from a 4 chamber cine view B-Delayed enhancement consistent with edema, scaring or fibrosis of the LV septum Heart 2005;91:582

31 Largest Published Series 34 Patients Followed for ~3 Years Clinical features: Heart failure 53% Heart transplantation 12% Thromboembolic events 24% Ventricular tachycardia 41% JACC 2000;36:

32 34 Patients Followed for 3 Years Many of these patients who presented with congestive heart failure had dyspnea and profound pulmonary edema with relatively preserved LVEFs and Doppler changes which were indicative of diastolic dysfunction.

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36 TTE, ECG and LV-gram From 58 YO Woman with Head Injury

37 Differential Diagnosis Acute Myocardial Infarction Cocaine-related ACS Myocarditis Pheochromocytoma Stress Cardiomyopathy

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39 Stress Cardiomyopathy This pathological process has many names Stress Cardiomyopathy Apical Dilation in the Absence of CAD Tako Tsubo Syndrome Broken Heart Syndrome

40 Transient LV Apical Dilation in the Absence of CAD Initially described in the Japanese literature. Tako Tsubo Syndrome = Octopus trap The syndrome consists of chest pain associated with ST-T abnormalities, moderate increases in cardiac markers, and a reversible apical wall motion abnormality in the absence of coronary artery disease. It is typically associated with emotional or mental stress. Bybee et al: Ann Intern Med 2004;141:

41 Classic Japanese Octopus Trap: Tako Tsubo

42 Transient LV Apical Dilation in the Absence of CAD The syndrome more often affects postmenopausal women. The in-hospital mortality rate seems to be low, as does the risk for recurrence.

43 Left Ventricular Apical Ballooning Cardiac MRI

44 Clinical Characteristics of 19 Patients with Stress Cardiomyopathy on Admission Wittstein, I. S. et al. N Engl J Med 2005;352:

45 Typical Electrocardiograms Obtained 24 to 48 Hours after Presentation in Four Patients with Stress Cardiomyopathy Wittstein, I. S. et al. N Engl J Med 2005;352:

46 Serial Echocardiographic Assessment of the Ejection Fraction in 19 Patients with Stress Cardiomyopathy Wittstein, I. S. et al. N Engl J Med 2005;352:

47 Ventriculographic Assessment of Cardiac Function and MRI Assessment of Myocardial Viability at Admission in a Patient with Stress Cardiomyopathy Wittstein, I. S. et al. N Engl J Med 2005;352:

48 Transient LV Apical Dilation in the Reversible balloon-like asynergy at the apex with hypercontraction of the basal segment of the ventricle was observed during the acute phase This disappeared during the chronic phase. The interval between these left ventriculograms from acute to chronic phase was 51 days. Absence of CAD

49 Plasma Catecholamine and Neuropeptide Levels Wittstein, I. S. et al. N Engl J Med 2005;352:

50 Transient LV Apical Dilation in the Absence of CAD The majority of patients survive this problem The only autopsy report is that of a patient who died of multiple organs system failure who also developed Takotsubo s Syndrome. The patient had no macroscopic signs of recent myocardial infarction or scars. Microscopic examination revealed normal myocardial tissue, except for some fatty infiltration. This observation suggests that acute myocarditis does not contribute to the etiology of this syndrome.

51 Diagnostic Criteria for Primary Transient Left Ventricular Apical Ballooning Major criteria 1. Reversible balloon-like left ventricular wall motion abnormality at the apex with hypercontraction of the basal segment 2. ST-T segment abnormalities on ECGs mimicking acute myocardial infarction Minor criteria 1. Physical or emotional stress as triggering factors 2. Limited elevation of cardiac markers 3. Chest pain

52 Modified Mayo Clinic Criteria Transient regional wall motion abnormality w/ or w/o apical involvement and a stressful trigger often but not always present. Absence of obstructive CAD or plaque rupture New ECG abnormalities or cardiac enzyme elevation Absence of pheochromocytoma or myocarditis