Peripartum Cardiomyopathy
Albert Mugisa, Deus Twinomugisha, ECP, Tracy Walczynski, MD, & Michael Schick, DO
Global Emergency Care, Mbarara University of Science and Technology, Uganda, Department of Emergency Medicine, Alice Springs Hospital, Northern Territory, Australia, & Department of Emergency Medicine, University of California, Davis
Clinical Presentation
History
Two sisters presented to the Emergency Department (ED) with concerns that their abdomens were increasing after giving birth. There was no associated infective symptoms or chest pain. Neither sister had a significant past medical history, specifically no hypertension. No allergies or regular medications were recorded.
| SISTER 1 | SISTER 2 |
|---|---|
| 29-year-old G3P3 Caesarean for obstructed labour two-months prior to presentation. Normal antenatal assessments. Reported development of abdominal distension, peripheral edema and paroxysmal nocturnal dyspnea during the post-partum period. | 28-year-old G3P3Spontaneous vaginal delivery one-month prior to presentation. Normal antenatal assessments. Developed shortness of breath on minimal exertion, associated with abdominal distension and peripheral edema during the post-partum period. |
Physical exam
| Sister | Blood pressure | Pulse | Respiratory Rate | Pulse Oximetry | Temperature |
|---|---|---|---|---|---|
| 1 | 120/65 | 105 | 30 | 99 | 36.2 |
| 2 | 106/90 | 127 | 35 | 95 | N/A |
N/A = not available
Sister two's pulse oximetry drops to low 90s when lying flat.
| Sister 1 | Sister 2 | |
|---|---|---|
| General | Appears well, no acute distress | Thin woman with obvious abdominal distension and moderate respiratory distress |
| HEENT | Mild conjunctival pallor | Mild conjunctival pallor |
| Neck | Jugulovenous distension,full ROM, no meningism. | Jugulovenous distension,full ROM, no meningism. |
| Cardiovascular | Mild, regular tachycardia, no murmur, rubs or gallops. | Moderate, regular tachycardic with diastolic murmur, no rubs or gallops . |
| Respiratory | Breathing comfortably on room air when seated, orthopnoea on lying supine. Diminished breath sounds and dullness to percussion at the RLL base, no wheezing, rales or crackles . | Increased work of breathing, tachypnea, accessory muscle use. Orthopnoea, unable to tolerate supine position. Diminished breath sounds bilateral base, and dullness to percuss bilaterally. |
| Abdomen | Grossly distended abdomen with fluid wave. Non-tender to palpation, no organomegaly palpable (limited by distension). | Tense, tender distension of abdomen, with fluid wave. |
| Musculoskeletal | Normal muscle tone | Normal muscle tone |
| Extremity | Peripheral edema +3, nil trauma, nil deformities. Strong, equal pulses upper and lower limbs. | Peripheral edema +3, nil trauma, nil deformities. Strong, equal pulses upper and lower limbs. |
| Neurologic | Alert and oriented x 4, no focal neurology findings. | Alert and oriented x 4, no focal neurology findings. |
| Skin | No rashes or lesions, pitting edema extending to mid-thigh. No sacral edema. | No rashes or lesions, pitting edema extending to lower abdomen. |
| Psychiatric | Linear thought process, good judgment, normal mood. | Linear thought process, good judgment, anxious. |
Imaging and Laboratory Information
Clinical Differential Diagnosis
- Peripartum Cardiomyopathy
- Idiopathic Dilated Cardiomyopathy
- Valvular Heart disease
- Myocarditis
- Pulmonary / Extra-Pulmonary Tuberculosis
- Meigs' Syndrome
Ultrasound Imaging Findings
Sister 1
Trans-thoracic echocardiogram, parasternal long view demonstrating global hypokinesis and severely reduced left ventricular ejection fraction.
Trans-thoracic echocardiogram, parasternal short view demonstrating global hypokinesis and severely reduced left ventricular ejection fraction.
Trans-thoracic echocardiogram, apical four chamber view demonstrating global hypokinesis and severely reduced left ventricular ejection fraction.
Apex of the left lung demonstrating B-lines indicating interstitial fluid.
Right upper quadrant view demonstrating a large pleural effusion and free fluid under the diaphragm.
Sagittal pelvic view demonstrating a large amount of free intra-abdominal fluid.
E-point Septal Separation measuring 1.47cm indicating a left ventricular systolic function of 37%.
Sister 2
Trans-thoracic echocardiogram, parasternal long view demonstrating global hypokinesis, severely reduced left ventricular ejection fraction and a small pericardial effusion.
Trans-thoracic echocardiogram, parasternal short view demonstrating global hypokinesis, severely reduced left ventricular ejection fraction and a small pericardial effusion.
Trans-thoracic echocardiogram, sub-xiphoid view demonstrating intra-abdominal free fluid, a small pericardial effusion, and global hypokinesis.
Right upper quadrant view demonstrating a large pleural effusion and free fluid under the diaphragm.
Right upper quadrant view of the abdomen with free fluid around the liver tip and in the hepato-renal recess.
Sagittal pelvic view with free fluid surrounding the uterus.
E-point Septal Separation measuring 2.12 cm indicating a left ventricular systolic function of 22%.
Differential Diagnosis Based on Imaging
- Peripartum Cardiomyopathy
- Tuberculosis - pulmonary and extra pulmonary
Clinical Course and/or Management
Both sisters were admitted to hospital for initiation of medical therapy. This included diuretics (furosemide, spironolactone), antiarrhythmics (digoxin) and angiotensin-enzyme inhibitors (captopril). Both sisters improved marginally and were discharged at day five. At discharge sister 1 had symptomatic hypotension with a blood pressure of 90/50 supine, so the diuretics were ceased. This presumably precipitated a re-presentation with shortness of breath at day 14, requiring a therapeutic paracentesis and re-introduction of diuretics.
Diagnosis
Peripartum Cardiomyopathy
Discussion
Peripartum Cardiomyopathy (PPCM) is commonly regarded as an idiopathic condition. Symptoms of congestive cardiac failure develop secondary to left ventricular systolic dysfunction. It is a diagnosis of exclusion requiring a thorough assessment and workup to ensure no alternate causative factors. Echocardiography is a key component of the diagnosis. There are several studies investigating the possibility of a genetic predisposition. This theory is of particular relevance to the sisters presented here. It is a rare occurrence to have the opportunity to investigate two sisters with the same symptomatology presenting simultaneously. Certainly, there is a higher incidence amongst women of African descent with studies suggesting having a 15.7-fold higher risk relative risk of PPCM.
The diagnostic criteria first proposed defines PPCM as a reduced left ventricular ejection fraction (LVEF) < 45% presenting towards the end of pregnancy or in the five months after delivery, in a woman without previously known structural heart disease. In patients here, the LVEF was estimated at 22% and 38% using E-Point Septal Separation (EPSS) measurements. The EPSS measures separation between the anterior mitral valve leaflet and septum in early diastole. LVEF is then extrapolated from this measurement using the formulae 75-(2.5×EPSS). Certainly, for the emergency clinican at the bedside, EPSS is easily taught and much faster to perform than complex echocardiographic assessment and well suited for the low resource health environment.
Formal echocardiography as a diagnostic tool is not readily accessible or affordable in resource limited countries. Point-of-Care Ultrasound (POCUS) was vital to guide the diagnosis and treatment of post-partum cardiomyopathy in this case.
References
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- Gentry MB, Dias JK, Luis A, Patel R, Thornton J, Reed GL. African-American Women Have a Higher Risk for Developing Peripartum Cardiomyopathy. Journal of the American College of Cardiology. 2010;55(7):654-9.
- Demakis GJ, Rahimtoola HS. Peripartum Cardiomyopathy. Circulation. 1971;44(5):964-8.
- McKaigney CJ, Krantz MJ, La Rocque CL, Hurst ND, Buchanan MS, Kendall JL. E-point Septal Separation: a Bedside Tool for Emergency Physician Assessment of Left Ventricular Ejection Fraction. The American Journal of Emergency Medicine. 2014;32(6):493-7.
- Silverstein JR, Laffely NH, Rifkin RD. Quantitative Estimation of Left Ventricular Ejection Fraction from Mitral Valve E-Point to Septal Separation and Comparison to Magnetic Resonance Imaging. The American Journal of Cardiology. 2006;97(1):137-40.