june 21, 2012 balderama-mendieta
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June 21, 2012Balderama-Mendieta
OBJECTIVES Identify pertinent findings from the history
and physical examination that would contribute to the diagnosis of peripartum cardiomyopathy
Provide a systematic approach in diagnosing patients with peripartum cardiomyopathyDetermine supportive diagnostic
examinationsArrive at a definitive diagnosis
Learn how to conservatively manage patients with peripartum cardiomyopathy
Patient Profile
JT 32 year-old , Female Single, Filipino, Roman
Catholic From Quezon City Admitted for the 1st time at our
institution on November 30, 2011
Patient Profile
Merchandiser Denies intake of alcoholic beverages Denies smoking and taking illicit
drugs
Patient Profile
Rents the 1st floor of a 4-storey studio type apartment
Sufficient source of water and electricity in the neighbourhood
Garbage collected twice a week
Chief Complaint
Difficulty of breathing of 6 days duration
Source and Reliability The patient herself with fair (70%)
reliability
TemporalProfile
6 da
ys P
TA
5 da
ys P
TA
4 da
ys P
TA
3 da
ys P
TA
2 da
ys P
TA
1 da
y PT
A
Admission
0
0.1
0.2
0.3
0.4
0.5
0.6
0.7
0.8
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1
Chart Title
Difficulty of Breath-ing
2 pillow orthopnea
Paroxysmal Nocturnal Dysp-neaEasy Fatigability
Elevated BP 170/40
TemporalProfile
6 da
ys P
TA
5 da
ys P
TA
4 da
ys P
TA
3 da
ys P
TA
2 da
ys P
TA
1 da
y PT
A
Admission
0
1
2
3
4
5
6
7
8
9
Chart Title
Difficulty of Breath-ing
2 pillow orthopnea
Paroxysmal Nocturnal Dysp-neaEasy Fatigability
Elevated BP 170/40
TemporalProfile
6 da
ys P
TA
5 da
ys P
TA
4 da
ys P
TA
3 da
ys P
TA
2 da
ys P
TA
1 da
y PT
A
Admission
0
1
2
3
4
5
6
7
8
9
Chart Title
Difficulty of Breath-ing
2 pillow orthopnea
Paroxysmal Nocturnal Dysp-neaEasy Fatigability
Elevated BP 170/40
TemporalProfile
6 da
ys P
TA
5 da
ys P
TA
4 da
ys P
TA
3 da
ys P
TA
2 da
ys P
TA
1 da
y PT
A
Admission
0
1
2
3
4
5
6
7
8
9
Chart Title
Difficulty of Breath-ing
2 pillow orthopnea
Paroxysmal Nocturnal Dysp-neaEasy Fatigability
Elevated BP 170/40
TemporalProfile
6 da
ys P
TA
5 da
ys P
TA
4 da
ys P
TA
3 da
ys P
TA
2 da
ys P
TA
1 da
y PT
A
Admission
0
2
4
6
8
10
12
14
16
Chart Title
Difficulty of Breath-ing
2 pillow orthopnea
Paroxysmal Nocturnal Dysp-neaEasy Fatigability
Elevated BP 170/40
TemporalProfile
6 da
ys P
TA
5 da
ys P
TA
4 da
ys P
TA
3 da
ys P
TA
2 da
ys P
TA
1 da
y PT
A
Admission
0
2
4
6
8
10
12
14
16
18
Chart Title
Difficulty of Breath-ing
2 pillow orthopnea
Paroxysmal Nocturnal Dysp-neaEasy Fatigability
Elevated BP 170/40
Past Medical History Unremarkable
Maternal History
Menstrual History Menarche at 16 y/o, regular, 28-day cycle,
3 days duration, moderately soaked, 3 pads per day, no dysmenorrhea
Obstetrical History: G2P1 (1011) G1- 2004, Spontaneous abortion G2- 2011, LFT male via NSD, delivered at
a lying-in-clinic by a mid-wife, the patient had regular pre-natal check-up
Gynecologic History History of pelvic infection, UTI at 16 3/7
weeks AOG of G2 (treated with Cefuroxime 500 mg/tab BID, resolved)
Sexual History Coitarche at 23 y/o, 2 SP, no post coital
bleeding or dyspareunia Contraceptives History
None
Family History
Hypertension, CVD, and asthma – paternal and maternal sides
Review of SystemsGeneral No significant weight loss, weight gain
Skin No lumps, no sores, no itching, no changes in color; no changes in hair or nails, no changes in size or color of moles
Head No headache, no head injury, no dizziness and no light-headedness
Eyes No icteric sclerae, no pale palpebral conjunctivae
Ears Unremarkable
Nose No frequent colds, no discharge, no itching, no nosebleed
Throat No dentures, no hoarseness, no dry mouth, no frequent sore throats
Neck No swollen glands, anterior neck mass, no lumps, no pain and stiffness
Review of Systems
Respiratory No cough, no hemoptysis, no dyspnea, no wheezing, no tuberculosis
Gastrointestinal
No dysphagia, no nausea and vomiting, no melena, no jaundice, no indigestion, no fatty food intolerance, no acholic stool, no changes in bowel movement
Urinary No polyuria, no nocturia; no hematuria, no retention, no bleeding
Review of SystemsGenitoreproductive
gravidum striae, linea nigra, gravid uterus
Musculoskeletal swelling both feet, no redness, no history of trauma
Psychiatric No nervousness, no tension, no mood changes, no depression, no memory change
Neurologic Unremarkable
Hematologic No anemia, no easy bruising or bleeding
Endocrine No excessive sweating, no excessive thirst or hunger
PHYSICAL EXAMINATION
Admitting Physical Examination
General Survey
Awake, alert, ambulatory, weak-looking, ectomorph, in cardiorespiratory distress
Vital Signs
BP 130/90; HR 109bpm; RR 34 cpm; 36.1C
Admitting Physical Examination
Anthropometrics Height: 157 cm Weight: 48 kg BMI: 19.5
HEENT Anicteric sclerae, pink palpebral conjunctivae, no tonsillopharyngeal congestion, no cervical lymphadenopathies, distended neck veins
Admitting Physical Examination
Chest/Lungs Equal chest expansion, dullness noted on both lower lung fields, decrease vocal fremitus on both lateral fields, decrease breath sounds on both lower lung field, noted with bibasal crackles
Admitting Physical Examination
Heart Adynamic precordium, tachycardic, irregular rhythm, distinct S1 and S2, PMI at 5th ICS, MCL, no murmurs, no S3 gallops
Abdomen Globular, soft abdomen, NABS, no tenderness, no palpable masses
Admitting Physical Examination
Extremities Grade 3/5 pulses on all extremities, Grade 2 bipedal edema, cold extremities, no cyanosis
MSE Intact
Cranial Nerves
Intact
SALIENT FEATURES
Salient Features
32-year-old female Recently delivered a full term
baby boy Difficulty of breathing Orthopnea Paroxysmal nocturnal dyspnea Easy fatigability Bipedal edema
Salient FeaturesEuthyroidIn cardiorespiratory distress BP 130/90; HR 109bpm; RR 25bpmBMI: 19.5Distended neck veinsSubcostal retractionsDullness noted on both lower lung fields
In cardiorespiratory distress BP 130/90; HR 109bpm; RR 34 bpm BMI: 19.5 Distended neck veins Dullness noted on both lower lung
fields Decrease vocal fremitus on both
lateral fields
Salient FeaturesEuthyroidIn cardiorespiratory distress BP 130/90; HR 109bpm; RR 25bpmBMI: 19.5Distended neck veinsSubcostal retractionsDullness noted on both lower lung fields
Decrease breath sounds on both lower lung field
Noted with bibasal crackles Tachycardic Irregular rhythm Grade 2 bipedal edema
ADMITTINGIMPRESSION
CONGESTIVE HEART FAILURE
PROBABLY SECONDARY TO PERIPARTUM CARDIOMYOPATHY
R/O PNEUMONIA, SEVERE PRE-ECLAMPSIA
CASE DISCUSSION
Harrisons 18th ed.
of:
Salient features
32-year-old female Recently delivered a full term baby
boy Difficulty of breathing Orthopnea Paroxysmal nocturnal dyspnea Easy fatigability Bipedal edema
of:
Salient features
In cardiorespiratory distress Distended neck veins Dullness noted on both lower
lung fields Decrease vocal fremitus on both
lateral fields
of:
Salient features Decrease breath sounds on both lower lung
field Noted with bibasal crackles Tachycardic Irregular rhythm Grade 2 bipedal edema
:
of:
Chest Xray
of:
Framingham Criteria MAJOR Paroxysmal
nocturnal dyspnea Neck vein distention Rales Radiographic
cardiomegaly Acute pulmonary
edema S3 gallop Increased central
venous pressure (>16 cm H2O at right atrium)
Hepatojugular reflux Weight loss >4.5 kg in
5 days in response to treatment
MINOR Bilateral ankle
edema Nocturnal cough Dyspnea on ordinary
exertion Hepatomegaly Pleural effusion Decrease in vital
capacity by one third from maximum recorded
Tachycardia (heart
rate>120 beats/min.)
of:
Peripartum Cardiomyopathy
Sliwa K, Hilfiker-Kleiner D, Petrie M, et.al. Current State of knowledge on aetiology, diagnosis, management, and therapy of peripartum cardiomyopathy: a position statement from the Heart Failure Association of the European Society of Cardiology Working Group on Peripartum Cardiomyopathy. European Journal of Heart Failure (2010) 12, 767–778
of:
Epidemiology
4% of all cardiomyopathies Incidence:
Geographic variations exist1 in 500–4000 in the USA1 in 1000 in South Africa1 in 300 in Haiti
Mielniczuk LM, Williams K, Davis DR, Tang AS, Lemery R, Green MS, Gollob MH, Haddad H, Birnie DH. Frequency of peripartum cardiomyopathy. Am J Cardiol 2006;97:1765–1768
of:
Epidemiology
CDC Pregnancy Related Mortality Surveillance 1991-1999Leading Causes of Maternal Mortality:
○ Embolism – 20%○ Hemorrhage – 17%○ Hypertension – 16%○ Peripartum Cardiomyopathy- 9%*
Mielniczuk LM, Williams K, Davis DR, Tang AS, Lemery R, Green MS, Gollob MH, Haddad H, Birnie DH. Frequency of peripartum cardiomyopathy. Am J Cardiol 2006;97:1765–1768
of:
Epidemiology
• 78% present within the first 4 months postpartum• Only 9% may present in the last month of pregnancy
Mielniczuk LM, Williams K, Davis DR, Tang AS, Lemery R, Green MS, Gollob MH, Haddad H, Birnie DH. Frequency of peripartum cardiomyopathy. Am J Cardiol 2006;97:1765–1768
of:
Etiology Etiology of this disorder is unclear. Proposed mechanisms:
nutritional deficienciesgenetic disordersviral or autoimmune etiologieshormonal problemsvolume overloadalcoholphysiologic stress of pregnancyunmasking of latent idiopathic dilated
cardiomyopathy
Fuster V, Alexander R, O’Rourke, et.al. Hurst’s THE HEART. Chapter 32: Myocarditis and Specific Cardiomyopathies. 12th Edition.
Clinical Presentations
Third heart sound (S3): 92% Fourth heart sound (S4) Tricuspid or mitral insufficiency
murmurs: 43% Displaced apical impulse: 72% Edema Rales Ascites Hepatomegaly Jugular venous distensionFuster V, Alexander R, O’Rourke, et.al. Hurst’s THE HEART. Chapter 32: Myocarditis and Specific Cardiomyopathies. 12th Edition.
Alogorithm
Sliwa K, Hilfiker-Kleiner D, Petrie M, et.al. Current State of knowledge on aetiology, diagnosis, management, and therapy of peripartum cardiomyopathy: a position statement from the Heart Failure Association of the European Society of Cardiology Working Group on Peripartum Cardiomyopathy. European Journal of Heart Failure (2010) 12, 767–778
Diagnostics
Electrocardiogramor
β-type Natriuretic Peptideand
Cardiac Imaging:EchocardiographyCardiac MRI
Sliwa K, Hilfiker-Kleiner D, Petrie M, et.al. Current State of knowledge on aetiology, diagnosis, management, and therapy of peripartum cardiomyopathy: a position statement from the Heart Failure Association of the European Society of Cardiology Working Group on Peripartum Cardiomyopathy. European Journal of Heart Failure (2010) 12, 767–778
Patient’s ECG
PPCM ECG
LVH: 66%NSSTTWC: 96%
Desai D, Moodley J, Naidoo D. Peripartum cardiomyopathy: experiences at King Edward VIII Hospital, Durban, South Africa and a review of the literature. Trop Doct 1995;25:118–123
BNP As a result of elevated LV end-diastolic
pressure due to systolic dysfunction, patients with PPCM commonly have an increased plasma concentration of B-type natriuretic peptide (BNP) or N-terminal pro-BNP (NT-proBNP).
38 patients with PPCM were compared with 21 healthy mothers post-partum: PPCM mothers: all had abnormal NT-proBNP
plasma levels (mean 1727.2 fmol/mL) Healthy mothers: mean 339.5 fmol/mL P < 0.0001
Sliwa K, Hilfiker-Kleiner D, Petrie M, et.al. Current State of knowledge on aetiology, diagnosis, management, and therapy of peripartum cardiomyopathy: a position statement from the Heart Failure Association of the European Society of Cardiology Working Group on Peripartum Cardiomyopathy. European Journal of Heart Failure (2010) 12, 767–778
Patient’s Echocardiography
PPCM Echocardiography
Most widely available imaging modality
Echocardiography should be repeated before patient discharge and at 6 weeks, 6 months, and annually to evaluate the efficacy of medical treatment
Sliwa K, Hilfiker-Kleiner D, Petrie M, et.al. Current State of knowledge on aetiology, diagnosis, management, and therapy of peripartum cardiomyopathy: a position statement from the Heart Failure Association of the European Society of Cardiology Working Group on Peripartum Cardiomyopathy. European Journal of Heart Failure (2010) 12, 767–778
PPCM Echocardiography
• Patient with persistent left ventricular dysfunction after pregnancy • Left ventricular and left atrial dilatation• There is a thrombus at the left ventricular apex
PPCM Echocardiography
Peripartum Cardiomyopathy(Post-partum)
Repeat Echo 6 weeks after delivery
Pathophysiology
Prolactin Cleavage and its role in PPCM
Previous studies have attributed PPCM to the following causesInflammationAutoimmune processesApoptosisImpaired cardiac microvasclatureProlactin cleavage
A study though by Hilfer-Kleiner et.al states that proteolytic cleavage of prolactin, brought upon most likely by peri/postpartum oxidative stress is the specific pathomechanism invloved in PPCMPro- apoptoticPro-inflammatory
16kDa prolactinDecrease in cardiac
densityInc. cardiac apoptosisAttenuates cardiac func.Promotes LV dilatation
Where does this happen? The process requires an acidic
environment (Cruz-Soto et al.) It is postulated that cleavage
occurs in acidic intracellular compartments
Lactotrophs being the most likely located in the adenohypophyseal area of the pituitary gland
Vasoinhibins Peptides that result from the cleavage of
full length prolactinAct on endothelial cells inhibiting proliferationReduce vasodilatation and vasopermeabilityPromote apoptosis-mediated vascular
regressionNatural inhibitors of the angiogenesis process
This is attributed to Cathepsin DDistributed in intracellular vesicles including
lysosomes and phagosomes
Lactotrophs Prolactin secreting cells, acidophilic Secretory granules have a pH of 5.3
and contain cathepsin D The cleavage process is inhibited by
Pepstatin A IN the case of PPCM with enough
production of 16kDa and stimulation for prolactin release there can be regulated release of 16kDa from the anterior pituitary lobe
Management Treatment is essentially the same with
dilated cardiomyopathy Goals:1. To reduce to amount of volume returning
to the heart (preload reduction)2. To decrease the resistance against which
the heart must pump (afterload reduction)
3. To increase the contractile force of the heart (inotropy).
Preload and afterload reductionLoop diuretics (caution in women with
preeclampsia)Hydralazine, nitrates and beta blockers
InotropyDigoxin (unless contraindicated)Dobutamine, dopamine, milrinone
Prophylaxis for thromboembolismLow dose heparin
Immunosuppressive therapy may be needed if peripartum cardiomyopathy is considered to be the result of myocarditis.
Patients should have a low sodium diet (≤ 4 gm) and fluid restriction (≤2L).
Activity should only be limited depending on the patient’s symptoms.
Cardiac transplantation and left ventricular assist devices are considered for women with progressive left ventricular dysfunction or deterioration despite medical therapy, however since most patients improve over time, surgical therapy should be delayed if possible.
Suggested Treatment Plan1. Institute ACE inhibitor therapy with
enalapril 5 mg twice daily and titrate up to a maximum dose of 20 mg twice daily, yet maintain SBP to 100 -110 mm Hg.
2. Start digoxin to achieve a serum level of 1 to 2 ng/dl.
3. Start diuretic therapy (furosemide 20 to 40 mg once daily) to control symptoms related to volume excess.
*Peripartum cardiomyopathy: A comprehensive reviewAmerican Journal of Obstetrics and Gynecology - Volume 178, Issue 2 (February 1998)
4. Start low-dose beta-blocker therapy (i.e., metoprolol 12.5 mg twice daily) and titrate for heart rate 80 to 100 beats/min.
5. Add additional vasodilator agents as needed to control systemic blood pressure (e.g., goal is systolic blood pressure 110 mm Hg).
6. Monitor ambulation for 24 to 48 hours.
7. Dietary consultation for fluid-restricted, low-salt diet.
8. Detailed patient education and counseling.
9. Referral to exercise rehabilitation program.
10. Vigilant follow-up to include measure of cardiac function within 3 to 6 months of treatment onset.
Prognosis Prognosis depends on return to normal left
ventricular function after the first episode of CHF.
In a study by Damaskis et al, they have observed that if the congestive cardiomyopathy persists after 6 months, it is likely irreversible and associated with a worse survival.
Recent studies have found that 30% of patients return to their baseline function after 6 months if given timely medical treatment.
Prognosis Mortality rate is 10% and the usual
causes of death include progressive heart failure, arrhythmia, or thromboembolism.
Patients who have peripartum cardiomyopathy are advised to avoid subsequent pregnancies due to concerns about the hearts inability to handle the increased cardiovascular workload during pregnancy.
Summary • PPCM –Dilated myopathy• Presents 36 weeks GA – 5
months Postpartum • 1:3000-4000 pregnancies• Increased maternal
mortality• Symptoms:
– Dyspnea, Edema, Orthopnea
• Dx: EKG and Echocardiogram
• Tx: Diuretics, B-blockers, ACEI, Anticoagulants
• Prognosis varies• Consult, consult, consult
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