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Ann Thorac Surg 2007;84:262-264
© 2007 The Society of Thoracic Surgeons

Case Reports

Extracorporeal Membrane Oxygenation in a Patient With Peripartum Cardiomyopathy

Hong Suk Yang, MDa, You Sun Hong, MDa, Se Joong Rim, MDb, Song Hyeon Yu, MDc,*

a Department of Thoracic and Cardiovascular Surgery, Yonsei University Medical College, Seoul, Republic of Korea
b Department of Cardiology, Yongdong Severance Hospital, Yonsei University Medical College, Seoul, Republic of Korea
c Department of Thoracic and Cardiovascular Surgery, Myongji Hospital, Kwandong University Medical College, KyungKi-Do, Republic of Korea

Accepted for publication February 20, 2007.

* Address correspondence to Dr Yu, Department of Thoracic and Cardiovascular Surgery, Myongji Hospital, Kwandong University Medical College, Dugyanggu, Goyangsi, KyungKi-Do, 412-270 Republic of Korea (Email: csysh1{at}hanmail.net).


   Abstract
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 Abstract
Introduction
 Comment
 References
 
An 18-year-old pregnant woman had cardiac failure and severe pulmonary edema developed immediately after the delivery of her baby. The patient’s respiratory distress was severe and her oxygen saturation was under 50%, despite full mechanical ventilatory support. Echocardiogram revealed an ejection fraction of 18%, and she was diagnosed with peripartum cardiomyopathy. Her vital signs were unstable when she received conventional treatment for acute heart failure. Extracorporeal membrane oxygenation was applied 3 hours after the patient was transferred to the emergency department. The patient was weaned from extracorporeal membrane oxygenation 28 hours afterward, and she was extubated 2 days after extracorporeal membrane oxygenation. The patient was discharged 12 days after admission.


   Introduction
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 Abstract
 Introduction
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Peripartum cardiomyopathy is a rare cause of heart failure that affects women late in pregnancy or in the early puerperium. Although initially described in 1849, it was not recognized as a distinct clinical entity until the 1930s [1]. Prognosis is dependent on the recovery of systolic function and mortality rates range from 7% to 50%.

An 18-year-old pregnant woman, who had been diagnosed with hypertension 3 years prior, was transferred to the emergency department for desaturation that occurred during a cesarean section in a nearby obstetric hospital. It was her first pregnancy, and she had no obstetric history before. The intrauterine period was 40 weeks and cephalopelvic disproportion was the reason for the cesarean section. Sinus tachycardia (160/min) and a high blood pressure (160/110 mm Hg) were noted, and her oxygen saturation was below 30%. Chest roentgenogram showed diffuse consolidation consistent with severe pulmonary edema (Fig 1). An initial arterial blood gas study showed severe respiratory acidosis. Mechanical ventilatory support was applied after endotracheal intubation.


Figure 1
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  		Fig 1. Severe pulmonary edema was shown in the chest x-ray taken at admission.

 
An echocardiogram was immediately used for cardiac evaluation. Severe global hypokinesia with an ejection fraction of 18% was present in the examination. The patient’s right ventricular systolic pressure was in the normal range and the size of the cardiac chambers and valves were also within normal values. The patient was transferred to the intensive care unit and listed with suspected postpartum cardiomyopathy.

Dobutamine and dopamine were used for inotropic support. Systolic blood pressure was maintained above 100 mm Hg after inotropic support, but the patient’s heart rate exceeded 160/min. An arterial blood gas study indicated severe hypoxemia, which was PO2 29 mm Hg with 44% saturation, despite the application of 100% oxygen. There was no improvement on the serial blood gas study for 2 hours.

We decided to apply an extracorporeal membrane oxygenation (ECMO) device 3 hours after the patient’s arrival to the emergency unit. Her left femoral artery and right femoral vein were used for percutaneous cannulation. Then a venoarterial bypass was initiated. Blood flow through the bypass circuit was increased to achieve satisfactory arterial oxygen saturation, and ventilator settings were decreased to minimize potential ventilator-induced lung injury.

The oxygen profile improved after the institution of ECMO, and the ventilator setting was decreased from 100% oxygen to 80% after 2 hours, and then to 60% after another 5 hours. Oxygen saturation was maintained above 90%. Dobutamine was tapered and norepinephrine was used for blood pressure control. Bypass flow rates were gradually reduced and by hour 28 of ECMO application, a trial period without ECMO was conducted. The patient was hemodynamically tolerable and oxygen saturation was kept above 95% with 60% oxygen. The ejection fraction was recovered up to approximately 45% in the echocardiogram, allowing treatment to proceed with decannulation.

Vaginal bleeding was prominent after anticoagulation treatment and the patient needed a transfusion. The activated clotting time was maintained at 150 to 200 seconds. Misoprostol and oxytocin were given to reduce vaginal bleeding, which stopped the day after decannulation. The fluid balance was positive during the ECMO period (approximately 18,000 cc) and then it was approximately 15,000 cc negative for 4 days without diuretics.

The patient was weaned from the ventilator and was extubated by day 4 of treatment. The patient’s vital signs were stable, and she had no respiratory distress or other complications related to heart failure. An echocardiogram revealed a decrease in the left ventricular end-diastolic dimension from 52 mm to 42 mm, and she showed improvement of her ejection fraction from 18% to 56%. The patient was transferred to the general ward the day after extubation. She was discharged on day 12 after admission (Fig 2).


Figure 2
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  		Fig 2. Chest x-ray taken during an outpatient visit (1 month after discharge).

 

   Comment
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References
 
Four criteria are needed to meet the definition of peripartum cardiomyopathy. First, is the requirement for the development of cardiac failure in the last month of pregnancy or within 5 months of delivery. Second, is the absence of an identifiable cause for cardiac failure. Third, the patient should not have recognizable heart disease prior to the last month of pregnancy. Finally, the patient must display left ventricular systolic dysfunction demonstrated by classic echocardiographic criteria, such as left ventricular ejection fraction with levels below 45% [2].

The incidence of peripartum cardiomyopathy (PPCM) is estimated to be 1 in 15,000 deliveries. No distinct hormonal disorder has been identified in patients with PPCM, even though estrogen, progesterone, and prolactin have significant effects on the cardiovascular system [3]. Many attempts have been made to clarify the cause of PPCM, but the cause still remains unknown.

Peripartum cardiomyopathy is rarely seen before 36 weeks of gestation. It usually affects the patient during the first 4 to 5 months postpartum. Patients with underlying cardiac disease have symptoms of heart failure develop during the second trimester of gestation because the greatest hemodynamic burden is imposed during this period [4]. Patients most commonly complain of dyspnea and other frequent symptoms include coughing, orthopnea, paroxysmal nocturnal dyspnea, and hemoptysis. Systemic and pulmonary emboli are more common in PPCM than in other types of cardiomyopathy [5].

Treatment of PPCM is similar to that for other types of heart failure. Diuretics, digoxin, beta-blockers, and afterload reduction are the treatments of choice. Anti-coagulation should be considered due to the hypercoagulable state of the patient. Heart transplantation is often the only acceptable treatment for women in whom more conventional therapy is not successful. Prognosis is dependent on the recovery of systolic function. Mortality from multiple small series ranges from 7% to 50%. Felker and colleagues [6] reported a mortality rate of 6% at 5 years and a transplant rate of 7% at 8.6 years post-treatment.

Extracorporeal life support outside the operating room was pioneered in the late 1960s; the first successful use of ECMO was reported in 1972 [7]. It was best studied as a treatment for respiratory failure in newborns. Its application has been extended to several other areas, such as patients with postoperative cardiogenic shock, patients experiencing severe myocardial dysfunction while undergoing cardiac catheterization, and patients with severe, reversible myocarditis or cardiomyopathy. Extracorporeal membrane oxygenation may act as a bridge to cardiac transplantation.

In the case of peripartum cardiomyopathy described in our study, the patient suffered from severe myocardial dysfunction, which was revealed by the echocardiography as an ejection fraction of 18%. Respiratory failure was severe due to cardiogenic pulmonary edema and heart failure was refractory to medical treatment. The patient showed severe desaturation, which would have been fatal without the early decision to apply extracorporeal membrane oxygenation. We decided on ECMO as the treatment of choice because the patient was healthy and young, and also due to the fact that survivors from PPCM mostly recover from significant decreases of ejection fraction.

The reported use of ECMO support has been predominantly for postcardiotomy cardiogenic shock and in cardiogenic shock related to acute myocardial infarction and acute myocarditis. Our experience with a female peripartum cardiomyopathy patient is likely a rare case of treating PPCM using ECMO. This case could also be an example of using an aggressive treatment modality in patients with severe PPCM who need prompt intervention due to their refractory response to inotropic and ventilatory support.


   References
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 Abstract
 Introduction
 Comment
 References
 

  1. Sliwa K, Fett J, Elkayam U. Peripartum cardiomyopathy Lancet 2006;368:687-693.[Medline]
  2. Pearson G, Veille JC, Rahimtoola S, et al. Peripartum cardiomyopathy: National Heart, Lung, and Blood Institute and Office of Rare Diseases (National Institutes of Health) workshop recommendations and review JAMA 2000;283:1183-1188.[Abstract/Free Full Text]
  3. Ro A, Frishman WH. Peripartum cardiomyopathy Cardiol Rev 2006;14:35-42.[Medline]
  4. Lee W. Clinical management of gravid women with peripartum cardiomyopathy Obstet Gynecol Clin North Am 1991;18:257-271.[Medline]
  5. Sliwa K, Fett J, Elkayam U. Peripartum cardiomyopathy Lancet 2006;368:687-693.[Medline]
  6. Felker GM, Jaeger CJ, Klodas E, et al. Myocarditis and long-term survival in peripartum cardiomyopathy Am Heart J 2000;140:785-791.[Medline]
  7. Lillehei CW. History of the development of extracorporeal circulationIn: Arensman RM, Cornish JD, editors. Extracorporeal life support. Boston, MA: Blackwell Scientific Publications; 1993. pp. 9-30.



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