Peripartum cardiomyopathy : case report

Correspondence to: Andrius Macas, Department of Ana­ esthesiology, Medical Academy, Lithuanian University of Health Sciences, Mickevičiaus 9, LT­44307 Kaunas, Lithuania. E­mail: andrius.macas@kaunoklinikos.lt Peripartum cardiomyopathy is an unusual and uncommon form of dilat­ ed cardiomyopathy that is often fatal to young women, the cause of which is unknown. Diagnostics is difficult and requires vigilance. The treatment does not differ from other forms of heart failure. Fetal outcome, however, is quite good. Maternal outcomes depend on 2–6 months recovery of the left ventricular function. We describe a previously asymptomatic patient who presented with pulmonary edema one day after caesarean section. In this case the solution was favorable to the patient. Complete recovery of the left ventricular function happened earlier than indicated in litera­ ture.


INTRODUCTION
Peripartum cardiomyopathy (PPCM) is an idio pathic cardiomyopathy, characterized by the heart failure, left ventricular systolic dysfunction to wards the end of pregnancy or WITHIN 5 months of delivery, when no other cause of the heart fail ure is found (1).There are no data about the inci dence of PPCM in Lithuania.In the USA, there is one case in 2 300 women giving birth and about 1 300 new cases a year (2).The incidence in South Africa is higher because of the AfricoAmerican women.
The exact pathogenetic mechanism is not fully understood.Several pathogenetic factors are as follows: inflammatory, infectious, genetic, autoim mune, oxidative stress.The diagnosis is confirmed on the basis of diagnostic criteria (3): a) develop ment of the heart failure during the last month of pregnancy or within 5 months of delivery; b) ab sence of an identifiable cause for the heart failure; c) absence of recognizable heart disease prior to the last month of pregnancy; d) left ventricular dysfunction determined during echocardiography with ejection fraction <45% (4).PPCM treatment is not different from acute and chronic heart failure treatment.Discontinuation of therapy is recommended only in case of recovery of the left ventricular function, gradually monitoring cardiac function and repeating the 2D cardiac ul trasound imaging (6).Recovery of the left ventricu lar function usually occurs within 2-6 months after diagnosis (7).The mortality rate ranges from 1% to 19% (8,9).Repeated risk of PPCM during subse quent pregnancy is 30-50%, if the left ventricular dysfunction remains.

CASE REPORT
A 38yearold woman was admitted to the Depart ment of Obstetrics and Gynecology for premature delivery and rupture of fetus membranes.It was her third pregnancy of 34 weeks.The patient had never had any heart disease.Her pregnancy was go ing well.She did not use any medication.The first two pregnancies and births were smooth.No bad habits.After arrival she complained about amniotic fluid flow and occasional, mild pain in the lower abdomen.Analysis performed in the Obstetrics and Gynecology Department showed increase in inflammatory markers: 19.37 × 10 9 /l leukocytosis, increased Creactive protein (CRP) -80.14 mg/l.However, there were no signs of clinical infection.Empirical antibacterial therapy was started (with cefuroxime).Gynecological examination revealed ruptured amniotic bubble, flow of clear amniotic fluid.Infection of the uterus was suspected there fore it was decided to perform the caesarean section.Lung maturation of premature fetus was started us ing glucocorticoids, tocolytic therapy with calcium (Ca) channel blockers was applied during lung maturation of the fetus.Clinical diagnosis: Gra viditas III (III) 34 hebdomas.Partus praematurus.Defluxus liqori amnii spontaneus praeterminalis.Cholestasis gravidarum.One day later, the planned cesarean section was performed under endotra cheal anesthesia after maturation of fetal lungs.After extubation, a short episode of hypoxaemia in the patient was observed (SpO 2 -97%-88%-94%), which was corrected with O 2 therapy mask over 6 l/min.Further treatment was applied in the In tensive Care Unit of the Obstetrics and Gyneco logy Department.The patient received infusion of 2 500 ml crystalloid, and duresis was only 500 ml over four hours.About 11 hours after surgery she started to complain of progressive shortness of breath at rest, chest discomfort, cough.During examina tion the overall condition was difficult: forced semisitting position.The patient was breathing spontaneously with supplied O 2 8 l/min through a face mask.Tachypnea was 28 rates / minute, SpO 2 87%.Vesicular breathing in lungs, on both sides, significant bilateral basal crackles.Sinus tachycardia was124 beats / minute, arterial blood pressure 114/82 mmHg.The cardiac auscultation showed normal first and second heart sounds.No significant cardiac murmurs were detected.She was afebrile.Metabolic acidosis determined during arterial blood gas analysis: pH -7.30; PCO 2 -33.1 mmHg; PO 2 -53.8 mm Hg; BE -(-9) mmol/l; HCO 3 -17.1 mmol/l.Ddimer was 7.8 mg/l, hypokalemia K 3.2 mmol/l, renal func tions, liver and clotting parameters were normal.Differentiation between pulmonary embolism and edema.Chest Xray showed a massive pulmo nary interstitial edema, heart size and shape un changed.Initial echocardiography showed poor left ventricular ejection fraction (LVEF -25%), middle and top parts were hypokinetic.Diastolic function was of a disordered relaxation type.Heart cavities were not dilated.Valves were in good con dition.There was no pulmonary hypertension.Vena cave superior was completely collapsed.The patient was hospitalized for further treatment to the Cardiac Intensive Care Unit because of the suddenly developed acute heart failure with pul monary edema.The electrocardiogram showed nonspecific Twave inversion in thoracic deriva tions.Elevated troponin I was 5.07-0.74µg/l and NTproBNP was 6 364 pg/l.Additional clinical di agnosis: Cardiomyophatia peripartum.Insuffici entia cordis acuta.Oedema pulmonum.
Pulmonary edema and heart failure treatment was applied with O 2 therapy 6 l/min through na sal cannula, intravenic nitrates, diuretics, preoral Bblockers (BAB) and angiotensin converting en zyme inhibitors (ACE), low molecular weight an ticoagulants (LMWH).The patient was allowed to breastfeed.She was treated in the Cardiologic In tensive Care Unit for 5 days.During the treatment, the left ventricular function significantly improved LVEF -25% → 38%.Pulmonary edema regressed.After two weeks the patient was discharged from hospital for outpatient treatment of the heart fail ure.A month later, repeated 2D echocardiography showed normalsized cardiac cavities, fully recov ered function of LVEF -60%.

DISCUSSIONS
In this case, peripartum cardiomyopathy was diag nosed upon exclusion of other reasons for the heart failure, according to the diagnostic criteria of peri partum cardiomyopathy (3).There were difficulties regarding diagnosis because PPCM belongs to the group of rare diseases.In this case, PPCM was cli nically observed on the first day after giving birth.According to literature, 93% of PPCM occur after delivery, of which 75% occur in the first month af ter delivery, and only 7% during the last trimester of pregnancy (10).
The risk of developing this disease is caused by the mother's age, number of pregnancies, multiple pregnancy, preeclamsia, gestational hypertension, oral tocolytic therapy with beta adrenergic ago nists.From patient's history it is known that she has never had heart and vascular diseases, but there was a number of risk factors, i. e. older age and a third pregnancy.
Symptoms and signs that can mimic heart failure are often attributed to normal pregnancy.Therefore, the disease is usually suspected only af ter pulmonary edema, arrhythmic, thromboembo lism complications and, in rare cases, by accident performing heart ultrasound.In this case, pulmo nary edema, which could be provoked by a general anesthesia and liquids overload, allowed suspect ing PPCM.It was diagnosed on the basis of the above mentioned diagnostic criteria (3), of which echocardiography is the most important to identify PPCM.Other tests such as the electrocardiogram and troponin I changes are considered to be non specific in this disease (6).
However, according to literature, such tests as high troponin I, plasma BNP levels, LVEF <30%, LV diastolic dimension <5.5 cm at the time of diag nosis, are factors of poor prognosis (6).Although in our case the patient had a number of such fac tors, the outcome was better than expected.
The patient was treated according to the treat ment guidelines of heart failure established by the European Society of Cardiologists (5).Attention was paid to the safety of medication to breast feeding mothers (11).Because of high incidence of thromboembolic complications typical of this disease, anticoagulant treatment with LMWH was prescribed for as long as the left ventricular func tion improved (LVEF >35%) (12).Thromboembol ic complications were not observed.
Breastfeeding in our clinic has caused a lot of discussions and opinions.The patient expressed a strong desire to breastfeed, so it was decided to al low moderate lactation.In this case it was a correct solution.Safirstein et al. showed that the recovery of the left ventricular function was statistically sig nificantly better for breastfeeding mothers (13).According to literature, in about 50% of all cases, the left ventricular function is restored within 2-6 months after diagnosis (7).In our case, the cor rect choice of treatment is proved by quick patient's recovery, total recovery of LV function within a month.

CONCLUSIONS
PPCM is a rare disease, the cause of which is un known, typical of women of childbearing age.Diag nostics is difficult and requires vigilance.The main objective of treatment is to reduce the symptoms of congestive heart failure.