Alas! It s Sponge Bob! Owen L. Sutarez, MD

Transcription

1 Alas! It s Sponge Bob! Owen L. Sutarez, MD

2 CASE J.A 46 year old male Married Roman Catholic presently residing at San Pedro Laguna admitted June 11, 2012

3 Chief Complaint: Shortness of Breath

4 History of Present Illness 7 mos PTA easy fatigability during exertion Consulted private physician, laboratory work up and chest xray was done which allegedly revealed heart enlargement. Rx: Digoxin 0.25 mg/tab OD Losartan 50 mg ½ tab OD Spironolactone 25 mg/ta OD L-carnitine tab OD. Condition apparently improved 4 months PTA Patient discontinued all medications He lost to follow-up.

5 History of Present Illness 2 weeks PTA difficulty of breathing and easy fatigability - - relieved by rest associated ADMITTED with minimal swelling of both lower extremities. Follow up with private physician was made wherein 2 DED and other laboratory examinations were requested. Previous medications resumed Three days PTA progressive difficulty of breathing apparently increase swelling of both lower extremities

6 Review of Systems: CNS: no headache, no focal weakness CVS: no chest pain, no palpitations Resp: occasional non productive cough GI: no vomiting, no diarrhea, no abdominal pain GUT: no dysuria, no hematuria Musculoskeletal: no muscle pain, no joint pain Hema: no bleeding manifestations

7 Past Medical History June 2011 diagnosed with Colon CA, stage III, treated with 12 cycles of chemotherapy using Oxaliplatin (Eloxatin) (-) HPN (-) DM (-) Asthma

8 Family Medical History unremarkable Personal Social History non-smoker occasional alcoholic beverage drinker denied allergy to foods and drugs

9 Physical Examination: General Survey: patient is conscious, coherent, not in cardiorespiratory distress Vital Signs: BP: 120/70 mmhg RR: 18 CR: 96 Temp: 36.6C SHEENT: Pink palpebral conjunctiva, no alar flaring, no naso-aural discharge, JVP at 10 cmh20 Chest/Lungs: Symmetrical chest expansion, no retractions, basal crackles on left lung field, decrease breath sounds on right mid to base lung fields. Heart: Adynamic precordium, no thrill, no heave, PMI at 5 th ICS, LMCL; normal rate and rhythm, no s3, no s4 Abdomen: flabby, normoactive bowel sound, soft, non tender Extremities: full and equal pulses, Grade 2 bipedal edema

10 Neurologic Exam: oriented to time, place and person Intact cranial nerves Sensory: 100% on all extremities Motor: 5/5 on all extremities DTR: ++ on all extremities

11 Based on initial findings, will you highly consider Oxaliplatin as a cause of possible Cardiomyopathy? A. Yes B. No

12

13 COURSE IN THE WARD

14 Upon BUN CBC: 4.2 Hgb (3.2 Admission mmol/l) Crea 82 Hct ( umol/l) Ionized Cal WBC ( mmol/l) Urinalysis: Magnesium Seg ( mmol/l) Potassium ph 6 Lymphos 3.8 ( mmol/l) Sodium CHON -Monos (-) 136 PLAN ( mmol/l) PT: SGPT CHO (-) 65 (21 72 U/L) S.G Test secs. ABG: Pus 1-3/hpf Control ph PTT: RBC 1-3/hpf pco2 38 CBC, Serum elec, PT, PTT, BUN, Crea, SGPT, UA, ABG, Few amorphous Test po urates secs CXR and 12 Lead ECG Control SO2 86% 13.4 Digoxin (Lanoxin) CEA (< 2.5) mg INR HCO3 OD,Losartan mg tab, ½ tab OD, Aldactone 25 mg/tab BID, % BE Activity Co-Enzyme 1 72% 1 tab TID and L- Carnitine 1 tab OD Total CHON 72 (63-82 g/l) Pulmonary and A/G Oncology Ratio 1.0 ( ) services for Co-management Albumin 36 (35 50 g/l) chest ultrasound with mapping, CEA Globulin 36 (15 35 g/l)

15 ECG : PR interval = 0.16 Sinus rhythm with occasional premature ventricular contractions Left Axis Deviation Left Atrial Enlargement Old Anterior Wall Infarct considered Non specific T Wave changes QRS = 0.06 QT interval = 0.36 Axis = -30

16 Chest X-ray Impression: Pleural Effusion, Right Presence of minimal pleural fluid on the left is not ruled out Magnified heart CXR Mapping Pleural Effusion, Right (approx 400cc fluid)

17 2DED LA = 50mm RA = 38mm EF = 27 LVMI = 166g/m2 RWT = 0.28 Estimated non compaction to compaction ratio = 2.5:1

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19 Asymptomatic individuals majority diagnosed with LVNC either during family screening or after an incidental diagnostic test such as an echocardiogram Koh YY, Seo YU, Woo JJ, et al: Familial isolated noncompaction of the ventricular myocardium in asymptomatic phase. Yonsei Med J 2004;45:

20 What is the diagnostic tool of choice for LV non compaction? A. 2DED B. MRI C. CT scan D. Coronary Angiogram

21 Diagnosis The diagnosis of LVNC is mostly based on the morphologic features of the LV. Echocardiography has been the routine initial noninvasive diagnostic test to detect LVNC and is still the diagnostic test of choice.

22 Echocardiography Typically, it shows a 2-layered appearance of the myocardium (an outer thin compact layer and a noncompact trabeculated thick endocardial layer) with deep intertrabecular spaces mostly near the left ventricular apex Systolic and diastolic function abnormalities may be seen frequently in the symptomatic patients.

23 LVNC Dilated Cardiomyopathy

24 What is the most common location of trabeculations according to the 16-segment leftventricular echocardiographic model? A. Inferior apical B. Inferior lateral C. Inferior septum D. Anterior apical

25 Location of trabeculations according to the 16-segment leftventricular echocardiographic model Diagnosis of left-ventricular non-compaction in patients with left-ventricular systolic dysfunction: European Heart Journal (2008) 29, ;doi:

26 1 st Hospital Day S O A P difficulty of breathing Swelling of lower extremities Comfortable Stable Vital Signs Frequent PVCs and nonsustained VTAC t/c Left Ventricular Non Compaction Right Pleural Effusion probably 2 Colon CA, stage III, s/p chemotherapy (2011) Amiodarone Drip MgSO4 and KCl Magnesium 0.77 ( mmol/l)

27 ECG Sinus Rhythm with occasional premature ventricular contractions Left Axis Deviation Left Atrial Enlargement Non-specific ST T wave changes PR interval = 0.16 QRS = 0.06 QT interval = 0.36 Axis = -30

28 What is the typical ECG findings of patient with LV non compaction? A. ST-T wave changes B. Ventricular arrhythmias C. Left bundle branch block D. All of the above

29 Electrocardiogram: include mostly ST-T wave changes, left ventricular hypertrophy,left bundle branch block (21%- 44%), complete atrioventricular block, atrial fibrillation (7%- 26%),ventricular ectopic beats, ventricular tachycardia (4.2%-30%), or ventricular fibrillation. Case reports of giant P waves and persistent atrial standstill with junctional rhythm have been published. Wolff Parkinson White pattern is more common in children and infrequent in adults.

30 What other non invasive test to confirm diagnosis of LV non compaction if 2DED is suboptimal? A. Cardiac Magnetic Resonance B. CT scan C. TEE D. Agitated saline bubble contrast

31 Cardiac magnetic resonance (CMR) imaging may be complimentary or even superior to echocardiography in establishing the diagnosis. Computed tomography (CT) has been found to be useful in a limited number of cases. Among invasive tests, contrast left ventriculography, video angioscopy, and endoscopy have been used to establish the diagnosis. Finally, a diagnosis of LVNC may be made or confirmed from an explanted heart, or at autopsy.

32 Diagnostic criteria for left-ventricular non-compaction LVNC is defined by a ratio of X/Y < 0.5 X = distance from the epicardial surface to the trough of the trabecular recess Y = distance from the epicardial surface to peak of trabeculation These criteria focus on trabeculae at the LV apex on the parasternal short axis and apical views, and on leftventricular free-wall thickness at end-diastole Chin TK, Perloff JK, Williams RG, et al. Isolated noncompaction of left ventricular myocardium: Circulation. 1990;82:

33 Diagnostic criteria for left-ventricular non-compaction (i)a two-layer structure, with a thin compacted layer and a thick non-compacted layer measured in end systole at the parasternal short-axis views LVNC is defined by a ratio of N/C >2 where N = non-compacted layer of myocardium C = compacted layer of myocardium (ii) Absence of co-existing cardiac structural abnormalities (iii) Numerous, excessively prominent trabeculations and deep intratrabecular recesses (iv) Recesses supplied by intraventricular blood on colour Doppler Jenni R, Oechslin E, Schneider J, Attenhofer Jost C, Kaufmann PA. Echocardiographic and pathoanatomical characteristics of isolated left ventricular non-compaction: a step towards classification as a distinct cardiomyopathy. Heart 2001;86:

34 Diagnostic criteria for left-ventricular non-compaction (i) More than three trabeculations protruding from the left-ventricular wall, apically to the papillary muscles, visible in a single image plane (ii) Intertrabecular spaces perfused from the ventricular cavity, visualized on colour Doppler imaging Stollberger C, Finsterer J, Blazek G. Left ventricular hypertrabeculation, noncompaction and association with additional cardiac abnormalities and neuromuscular disorders. Am J Cardiol 2002; 90:

35 2 nd to 3 rd Hospital Day S O A P DOB relieved by rest Swelling of lower extremities Stable Vital Signs Pleural Fluid of 600cc with minimal Pericardial effusion Left Ventricular Non Compaction Right Pleural Effusion probably 2 Colon CA, stage III, s/p chemotherapy (2011) Low Salt Low fat Chest UTZ done Thoracentesis with Pleural Analysis done DOB resolved after Thoracentesis Weaned from O2

36 4 th Hospital Day S O A P Asymptomatic Able to ambulate Stable VS Left Ventricular Non Compaction Right Pleural Effusion probably 2 Colon CA, stage III, s/p chemotherapy (2011) s/p Thoracentesis DISCHARGED Home Meds: Amiodarone 200 mg tab, 1 tab BID, Digoxin (Lanoxin) 0.25 mg tab OD, Losartan 50mg tab, ½ tab OD, Aldactone 25 mg/tab BID, Trimezatidine 35 mg/tab, 1 tab BID, Clopidogrel 75 mg/tab, 1 tab OD and Co- Enzyme 1 tab TID

37 Final Diagnosis Left Ventricular Non Compaction Right Pleural Effusion probably 2 Colon CA, stage III, s/p chemotherapy (2011) s/p Thoracentesis

38 What is the other name of LVNC? A. Spongy Myocardium B. LV hypertrabeculations C. Sponge Bob s Heart D. A & B

39 Left ventricular noncompaction (LVNC) Also known as spongy myocadium or LV hypertrabeculations spongy appearance of the myocardium increased trabeculation and deep intertrabecular recesses that communicate with the ventricles. The first case of isolated LVNC was reported by Engberding and Bender 25 years ago. In 2006, LVNC was classified as a primary cardiomyopathy of genetic origin by the American Heart Association (AHA).

40 Epidemiology The true prevalence of LVNC remains unclear may present at any age from infancy to older than 94 years. affect more males than females Left ventricular noncompaction may often go undiagnosed or misdiagnosed as another type of cardiomyopathy, most often hypertrophic or dilated cardiomyopathy.

41 Genetics Different genes found to be associated with LVNC are taffazin, β-dystrobrevin (DTNA), Cypher/ZASP (LDB3), lamin A/C (LMNA), SCN5A, MYH7, and MYBPC3. There is also significant overlap in the phenotypes of the genetically mediated cardiomyopathies. Thus, LVNC can occur with dilated or hypertrophic cardiomyopathy. the Heart Failure Society of America practice guidelines recommend clinical screening of all first degree relatives of affected patients for LVNC.

42 Anatomic pathology The trabeculated endocardial layer of normal hearts is never thicker than the compact myocardium. In LVNC, the trabeculated endocardial layer is at least 2 times thicker than the compacted layer. Trabeculations are rare in the basal segments of the ventricle.

43 Clinical presentation Symptomatic presentation of patients with isolated LVNC varies widely with some patients remaining asymptomatic for many years, whereas others develop symptoms very early in childhood. Asymptomatic individuals may be diagnosed with LVNC either during family screening or after an incidental diagnostic test such as an echocardiogram. One group of investigators reported associated neuromuscular disorders and facial dysmorphism in more than 50% of patients with LVNC.

44 What is the least probable cardiac events of patient with LV non compaction? A. Left ventricular systolic dysfunction B. Arrhythmias C. Thromboembolic complications D. Coronary artery disease

45 Symptoms are mainly due to left ventricular systolic dysfunction, arrhythmias, and thromboembolic complications. Dyspnea seems to be the most common presenting symptom. Other major symptoms include chest pain, palpitations, syncope, cerebrovascular accidents, and other systemic or pulmonary embolic complications. Sudden death is not uncommon in this group because of malignant ventricular arrhythmias.

46 Management: Heart Failure Management Arrhythmias Thromboembolic complications

47 Heart failure Conventional therapy per the American College of Cardiology (ACC)/AHA guidelines is recommended for patients with LVNC and heart failure. β-blockers, angiotensin-converting enzyme inhibitors, and diuretics are the main stay of therapy in symptomatic patients with LV systolic dysfunction. Digitalis, aldosterone inhibitors, and vasodilators are also used as needed.

48 When heart failure progresses despite maximum medical therapy, device-based therapy has been recommended. Cardiac resynchronization therapy (CRT) with or without an implantable cardioverter defibrillator (ICD) is a class I recommendation for those patients with: symptomatic heart failure NYHA functional class III ambulatory class IV while on optimal medical therapy left ventricular ejection fraction less than 35% and QRS duration more than 0.12 seconds.

49 Thromboembolic complications: Systemic thromboembolic complications are the third major complication in LVNC. In the presence of AF and left ventricular systolic dysfunction, the thromboembolic risk is high. Systemic embolic events including cerebrovascular accidents, transient ischemic attacks, embolism to coronary, and superior mesenteric arteries have been reported. Routine anticoagulation with warfarin has therefore been recommended by few, whereas others reported using anticoagulation therapy in high-risk individuals with LVNC.

50 Although left ventricular thrombus formation in noncompaction is rare according to the review of 22 articles by Stollberger et al, reported incidence of thromboembolic complications ranges from 5% to 38%.

51 Arrhythmias Supraventricular arrhythmias Atrial fibrillation is a common arrhythmia in patients with LVNC, with a reported incidence between 7% and 26%. There is no specific treatment for patients with LVNC, and reported drug therapy included digoxin, β-blockers, calcium channel blockers, and amiodarone. Radiofrequency ablation has been successful in a case with atrioventricular nodal reentry tachycardia. Other supraventricular arrhythmias may also be treated using standard recommended therapy.

52 Ventricular arrhythmias: Incidence of ventricular arrhythmias in patients with LVNC has been reported to be as high as 62% to as low as 6%. There are many case reports on ICD implants in patients with LVNC for ventricular arrhythmias both for primary and secondary prevention of sudden death with favorable results. According to the Device Based Therapy guidelines, implantation of ICD for the prevention of sudden death in patients with LVNC is a class IIb recommendation. Medical therapy of ventricular arrhythmias in LVNC is the same as for other patients with ventricular arrhythmias.

53 Who among group of patient with LVNC had poor prognosis? A. Atrial Fibrillation B. Dilated LA >40 mm C. EF < 31% D. All of the above

54 Prognosis: the prognosis of asymptomatic individuals with LVNC seems to be much better than those with symptoms. Left ventricular ejection fraction less than 31% has been reported to have a 71% sensitivity and 90% specificity to predict major adverse events left atrial dilatation more than 40 mm is also associated with poor prognosis.

55 Prognosis: Another group reported 22% mortality during a 51- month follow-up among patients with: advanced age associated neuromuscular disorders heart failure with dilated LV, and decreased ejection fraction were all associated with higher mortality. Atrial fibrillation with or without neuromuscular disease was associated with poor prognosis.

56 It should be noted that prognosis seems to be more favorable in the recent compared with the early reports. This may be related to : (1) aggressive medical management of patients with heart failure, arrhythmias, and thromboembolic events (2) better and more easily available diagnostic testing resulting in a broader spectrum of patients, which includes asymptomatic individuals with the morphologic diagnosis of LVNC. (3) the patient groups in the later reports include both symptomatic and asymptomatic individuals with much better prognosis than in the earlier reports, which included mostly symptomatic patients with some degree of complications.

57 Future Plans Serial follow-up studies of affected individuals might help elucidate the changing morphology noted after medical therapy or biventricular pacing. Genetic testing of the most clinically affected individuals, echocardiographic, or CMR screening first-degree relatives obtaining family history for at least 3 generations The association of neuromuscular diseases and LVNC also needs further research to identify the mechanisms involved in such association

58 Are you satisfied with our diagnosis as a case of LV non compaction? A. Yes B. No

59 THANK YOU!!!