Author Information
Amin K*, Shende D**, Dhokia T***, Chauhan AR****.
(* Third Year Resident, ** Assistant Professor, *** Second Year Resident, **** Additional Professor, Department of Obstetrics and Gynecology, Seth G S Medical College and KEM Hospital, Mumbai, India.)
Abstract
Cardiomyopathy is a life threatening form of cardiac failure which usually occurs in late pregnancy or early peurperium. The incidence of cardiomyopathy in pregnancy is rare and seen in 1:1485 to 4000 live births.[1,2] The chances of recurrence in subsequent pregnancy is around 21-80%.[3] We present a case of recurrent peripartum cardiomyopathy despite rapid return of normal cardiac function after the first pregnancy.
Introduction
Cardiomyopathy is characterized by its rapid clinical course and probability of spontaneous recovery. There is a risk of recurrence of cardiomyopathy and even death in subsequent pregnancies if the ejection fraction is persistently reduced. However if ventricular size and function have returned to normal in the postpartum period, the risk of recurrence is low.
Case Report
A 34 years old patient with multiple high risk factors and bad obstetric history (BOH) was referred to our tertiary care center at 34 weeks of gestation for evaluation of persistent breathlessness. She was a known case of type 2 diabetes mellitus. She was obese (weight 109kgs), had BOH (Gravida 7, Para1, with one living issue and 5 spontaneous abortions) with one previous full term lower segment cesarean section in view of transverse lie, with gestational hypertension in this pregnancy. She was on injection insulin and anti-hypertensive drugs. She presented to us with dyspnea on exertion (NYHA II), palpitations and decreased urine output since 15 days.
In the present pregnancy her general examination findings were suggestive of raised blood pressure of 140/90 mm of Hg with bilateral pitting pedal edema grade III. On abdominal examination, uterus was over-distended, abdominal wall edema was present, with single live fetus in longitudinal lie with cephalic presentation, no uterine activity, Pfannensteil scar of previous cesarean section with no scar tenderness. On per vaginal examination cervical os was closed.
Her hematological investigations were normal. Her 2-D echocardiogram done elsewhere 1 month prior to presentation in view of breathlessness was within normal limits. Ultrasound was suggestive of polyhydramnios with an amniotic fluid index of > 25 cm. After all other investigations, therapeutic amniocentesis was done as no other cause was found for her breathlessness; around 1 L of amniotic fluid was drained but patient was still symptomatic.
Hence cardiology opinion was sought and 2-D echocardiogram was done which was suggestive of left ventricular ejection fraction of 30% with moderate mitral regurgitation, tricuspid regurgitation and severe pulmonary hypertension. Diagnosis of peripartum cardiomyopathy was made. The patient was shifted to cardiac intensive care unit and was started on digoxin and intravenous frusemide drip along with insulin and antihypertensive drugs. While explaining to the patient about her heart condition, she revealed that in previous pregnancy she had peripartum cardiomyopathy for which she was managed in intensive care unit; she did not reveal this history as she thought the problem had been resolved.
After stabilization in ICU, emergency lower segment cesarean section with copper T insertion was done under general anesthesia in view of severe pulmonary hypertension and moderate maternal condition. Female child of 3.4 kg was delivered with good Apgar score. Patient was monitored in cardiac ICU. Her postoperative recovery was uneventful and she was discharged on day 7.
Discussion
In 1971, Demakis and colleagues first defined peripartum cardiomyopathy as idiopathic heart failure which occurs in the absence of any preexisting heart disease in the last month of pregnancy or during the first 5 months postpartum.[4] There are multiple risk factors for cardiomyopathy such as maternal age >35yrs, gestational hypertension, preeclampsia, multiple pregnancy, multiple gestations, obesity, and family history.
Chances of recurrence in subsequent pregnancy is around 21-80%. The risk of recurrence of cardiomyopathy is highest in patients who have persistent cardiac dysfunction. However the risk is lowest in those whose cardiac functions have been returned to normal & this can be detected by performing dobutamine stress test. Recurrence has also been reported in patients whose ventricular size and function have returned to normal as in our case.[5,6] They usually present with dyspnea on exertion, cough, orthopnea and paroxysmal nocturnal dyspnea and often mimic left ventricular failure (LVF). Nonspecific symptoms like palpitations, fatigue, malaise and abdominal pain may be present in 50% of cases.[5,7] Gallop rhythm, engorged neck veins, tachycardia and pedal edema are commonly found.
Diagnosis can be established by excluding common causes of cardiac failure such as toxins, infection, and ischemic, metabolic or valvular heart disease and it requires high degree of suspicion. Many features of cardiomyopathy resembles that of advanced pregnancy making the diagnosis difficult for doctor.[1] Electrocardiogram, chest radiograph and Doppler echocardiograph are most commonly used diagnostic modalities. Doppler echocardiography is the gold standard diagnostic tool for assessing the severity and prognosis of the disease. Criteria used to establish diagnosis are: left ventricular ejection fraction (LVEF) 45% or M-mode fractional shortening <30% (or both) and end-diastolic dimension >2.7 cm/m2. Common differential diagnoses include accelerated hypertension, preeclampsia, idiopathic cardiomyopathy, pulmonary embolism, anemia and thyrotoxicosis.[8] Complications such as arrythmias, thromboembolism, multiorgan failure, increased incidence of abortion (4-25%), premature delivery (11-50%), low birth weight and small for date babies, intrauterine growth retardation and fetal deaths are associated with it.[9] Congenital fetal anomalies can occur in 4-6% of cases.
Digoxin, diuretics, vasodilators, β blockers and anticoagulants are used for routine medical management. Immunosuppressive drugs, immunoglobulin and pentoxifylline can be considered in resistant cases. Severe cases may need intensive management, including mechanical circulatory support and heart transplant.
A multidisciplinary approach involving an obstetrician, cardiologist, anesthesiologist and neonatologist may be required for management of such patients. Induction of delivery should be considered if a patient's condition worsens despite maximal medical management.[5,6] Regional techniques are safer for both labor analgesia and anesthesia. Second stage of labor should be cut short using outlet forceps or vacuum to minimize cardiac stress. Cesarean section to be considered for obstetric indications and in situations were normal delivery can deteriorate further condition of patient as in our case. Following delivery, these patients should be monitored in an intensive care unit for prompt detection and management of uterine autotransfusion-induced pulmonary edema.[10,11]. Mortality rate is around 50%.[6] Future pregnancy is better avoided in patients with persistent cardiac failure.
References
Amin K*, Shende D**, Dhokia T***, Chauhan AR****.
(* Third Year Resident, ** Assistant Professor, *** Second Year Resident, **** Additional Professor, Department of Obstetrics and Gynecology, Seth G S Medical College and KEM Hospital, Mumbai, India.)
Abstract
Cardiomyopathy is a life threatening form of cardiac failure which usually occurs in late pregnancy or early peurperium. The incidence of cardiomyopathy in pregnancy is rare and seen in 1:1485 to 4000 live births.[1,2] The chances of recurrence in subsequent pregnancy is around 21-80%.[3] We present a case of recurrent peripartum cardiomyopathy despite rapid return of normal cardiac function after the first pregnancy.
Introduction
Cardiomyopathy is characterized by its rapid clinical course and probability of spontaneous recovery. There is a risk of recurrence of cardiomyopathy and even death in subsequent pregnancies if the ejection fraction is persistently reduced. However if ventricular size and function have returned to normal in the postpartum period, the risk of recurrence is low.
Case Report
A 34 years old patient with multiple high risk factors and bad obstetric history (BOH) was referred to our tertiary care center at 34 weeks of gestation for evaluation of persistent breathlessness. She was a known case of type 2 diabetes mellitus. She was obese (weight 109kgs), had BOH (Gravida 7, Para1, with one living issue and 5 spontaneous abortions) with one previous full term lower segment cesarean section in view of transverse lie, with gestational hypertension in this pregnancy. She was on injection insulin and anti-hypertensive drugs. She presented to us with dyspnea on exertion (NYHA II), palpitations and decreased urine output since 15 days.
In the present pregnancy her general examination findings were suggestive of raised blood pressure of 140/90 mm of Hg with bilateral pitting pedal edema grade III. On abdominal examination, uterus was over-distended, abdominal wall edema was present, with single live fetus in longitudinal lie with cephalic presentation, no uterine activity, Pfannensteil scar of previous cesarean section with no scar tenderness. On per vaginal examination cervical os was closed.
Her hematological investigations were normal. Her 2-D echocardiogram done elsewhere 1 month prior to presentation in view of breathlessness was within normal limits. Ultrasound was suggestive of polyhydramnios with an amniotic fluid index of > 25 cm. After all other investigations, therapeutic amniocentesis was done as no other cause was found for her breathlessness; around 1 L of amniotic fluid was drained but patient was still symptomatic.
Hence cardiology opinion was sought and 2-D echocardiogram was done which was suggestive of left ventricular ejection fraction of 30% with moderate mitral regurgitation, tricuspid regurgitation and severe pulmonary hypertension. Diagnosis of peripartum cardiomyopathy was made. The patient was shifted to cardiac intensive care unit and was started on digoxin and intravenous frusemide drip along with insulin and antihypertensive drugs. While explaining to the patient about her heart condition, she revealed that in previous pregnancy she had peripartum cardiomyopathy for which she was managed in intensive care unit; she did not reveal this history as she thought the problem had been resolved.
After stabilization in ICU, emergency lower segment cesarean section with copper T insertion was done under general anesthesia in view of severe pulmonary hypertension and moderate maternal condition. Female child of 3.4 kg was delivered with good Apgar score. Patient was monitored in cardiac ICU. Her postoperative recovery was uneventful and she was discharged on day 7.
Discussion
In 1971, Demakis and colleagues first defined peripartum cardiomyopathy as idiopathic heart failure which occurs in the absence of any preexisting heart disease in the last month of pregnancy or during the first 5 months postpartum.[4] There are multiple risk factors for cardiomyopathy such as maternal age >35yrs, gestational hypertension, preeclampsia, multiple pregnancy, multiple gestations, obesity, and family history.
Chances of recurrence in subsequent pregnancy is around 21-80%. The risk of recurrence of cardiomyopathy is highest in patients who have persistent cardiac dysfunction. However the risk is lowest in those whose cardiac functions have been returned to normal & this can be detected by performing dobutamine stress test. Recurrence has also been reported in patients whose ventricular size and function have returned to normal as in our case.[5,6] They usually present with dyspnea on exertion, cough, orthopnea and paroxysmal nocturnal dyspnea and often mimic left ventricular failure (LVF). Nonspecific symptoms like palpitations, fatigue, malaise and abdominal pain may be present in 50% of cases.[5,7] Gallop rhythm, engorged neck veins, tachycardia and pedal edema are commonly found.
Diagnosis can be established by excluding common causes of cardiac failure such as toxins, infection, and ischemic, metabolic or valvular heart disease and it requires high degree of suspicion. Many features of cardiomyopathy resembles that of advanced pregnancy making the diagnosis difficult for doctor.[1] Electrocardiogram, chest radiograph and Doppler echocardiograph are most commonly used diagnostic modalities. Doppler echocardiography is the gold standard diagnostic tool for assessing the severity and prognosis of the disease. Criteria used to establish diagnosis are: left ventricular ejection fraction (LVEF) 45% or M-mode fractional shortening <30% (or both) and end-diastolic dimension >2.7 cm/m2. Common differential diagnoses include accelerated hypertension, preeclampsia, idiopathic cardiomyopathy, pulmonary embolism, anemia and thyrotoxicosis.[8] Complications such as arrythmias, thromboembolism, multiorgan failure, increased incidence of abortion (4-25%), premature delivery (11-50%), low birth weight and small for date babies, intrauterine growth retardation and fetal deaths are associated with it.[9] Congenital fetal anomalies can occur in 4-6% of cases.
Digoxin, diuretics, vasodilators, β blockers and anticoagulants are used for routine medical management. Immunosuppressive drugs, immunoglobulin and pentoxifylline can be considered in resistant cases. Severe cases may need intensive management, including mechanical circulatory support and heart transplant.
A multidisciplinary approach involving an obstetrician, cardiologist, anesthesiologist and neonatologist may be required for management of such patients. Induction of delivery should be considered if a patient's condition worsens despite maximal medical management.[5,6] Regional techniques are safer for both labor analgesia and anesthesia. Second stage of labor should be cut short using outlet forceps or vacuum to minimize cardiac stress. Cesarean section to be considered for obstetric indications and in situations were normal delivery can deteriorate further condition of patient as in our case. Following delivery, these patients should be monitored in an intensive care unit for prompt detection and management of uterine autotransfusion-induced pulmonary edema.[10,11]. Mortality rate is around 50%.[6] Future pregnancy is better avoided in patients with persistent cardiac failure.
References
- Pearson GD, Veille JC, Rahimtoola S, Hsia J, Oakley CM, Hosenpud JD, et al. Peripartum cardiomyopathy: National Heart, Lung and Blood Institute and Office of Rare Diseases (National Institutes of Health) workshop recommendation and review. JAMA 2000;283:1183–1188.
- Whitehead SJ, Berg CJ, Chang J. Pregnancy-related mortality due to cardiomyopathy: United States, 1991-1997. Obstet Gynecol 2003;102:1326–1331.
- Fett JD, Carraway RD, Dowell DL, King ME, Pierre R. Peripartum cardiomyopathy in the Hospital Albert Schweitzer District of Haiti. Am J Obstet Gynecol 2002;186:1005–1010.
- Demakis JG, Rahimtoola SH, Sutton GC, Meadows WR, Szanto PB, Tobin JR, et al. Natural course of peripartum cardiomyopathy. Circulation. 1971;44:1053–1061.
- Sliwa K, Fett J, Elkayam U. Peripartum cardiomyopathy. Lancet. 2006;368:687–693.
- Ro A, Frishman WH. Peripartum cardiomyopathy. Cardiol Rev 2006;14:35–42.
- Sliwa K, Skudicky D, Bergemann A, Candy G, Puren A, Sarelil P. Peripartum cardiomyopathy: analysis of clinical outcome, left ventricular function, plasma levels of cytokines and Fas/Apo-1. J Am Coll Cardiol 2000;35:701–705.
- Ray P, Murphy GJ, Shutt LE. Recognition and management of maternal cardiac disease in pregnancy. Br J Anaesth 2004;93:428–439.
- Elkayam U, Akhter MW, Singh H, Khan S, Bitar F, Hameed A, et al. Pregnancy-associated cardiomyopathy: clinical characteristics and a comparison between early and late presentation. Circulation 2005;111:2050–2055.
- Task Force on the Management of Cardiovascular Diseases During Pregnancy of the European Society of Cardiology. Expert consensus document on management of cardiovascular diseases during pregnancy. Eur Heart J 2003;24:761–781.
- Ray P, Murphy GJ, Shutt LE. Recognition and management of maternal cardiac disease in pregnancy. Br J Anaesth 2004;93:428–439.
Amin K, Shende D, Dhokia T, Chauhan AR. Successful Management Of Recurrent Peripartum Cardiomyopathy. JPGO 2015. Volume 2 No. 12. Available from: http://www.jpgo.org/2015/12/successful-management-of-recurrent.html