Peripartum cardiomyopathy

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Synonyms and Keywords: Meadows syndrome

Overview

Peripartum cardiomyopathy (PPCM) is a form of dilated cardiomyopathy that is defined as a deterioration in cardiac function presenting between the last month of gestation and up to five months post-partum. As with other forms of dilated cardiomyopathy, PPCM involves decrease of the left ventricular ejection fraction with associated congestive heart failure and increased risk of atrial and ventricular arrhythmias and even sudden cardiac death. The definition of PPCM includes four criteria, three clinical and one echocardiographic:[1] PPCM occurs during last month of pregnancy or first five months after delivery, absence of an identifiable cause for cardiac failure, absence of heart disease prior to last five months of pregnancy, echocardiographic criteria -severe left ventricular systolic dysfunction, demonstrated by ejection fraction less than 45% or M-mode fractional shortening less than 30%(or both) and left ventricular end-diastolic dimension >2.7cm/m².

Historical Perspective

  • [Disease name] was first discovered by [scientist name], a [nationality + occupation], in [year] during/following [event].
  • In [year], [gene] mutations were first identified in the pathogenesis of [disease name].
  • In [year], the first [discovery] was developed by [scientist] to treat/diagnose [disease name].

Pathophysiology

The etiology of peripartum cardiomyopathy is not clearly known. Research suggests that cardiac angiogenic imbalance may play a role in peripartum cardiomyopathy.

Cardiac Angiogenic Imbalance and Peripartum Cardiomyopathy

Researchers indicated a two hit combination as the cause of peripartum cardiomyopathy. This includes:[2]

  1. Systemic anti-angiogenic signals during late pregnancy like elevated sFTL1 from the placenta which binds to and neutralizes soluble members of the VEGF(vascular Endothelial Growth Factor) family , elevated prolactin from the pituitary which on inappropriate cleavage by reactive oxygen species (ROS) forms a potent anti-angiogenic 16-kDa form, and excess angiotensin II signalling.
  2. A host susceptibility caused by
  • Insufficient local pro-angiogenic defenses in the heart. This includes:
  • A decrease in the transcriptional coactivator PGC-1α. PGC-1α drives mitochondrial biogenesis, regulates angiogenic programming and increases ROS scavenging.
  • Abnormal or absent STAT3. STAT3 is responsible for protection against ROS production.
  • Genetically predisposed
  • Immune activated
  • viral infection induced
  • auto-antibody induced
  • Inflammatory states of the heart induced.

PGC-1α knockout mice (mice in which all or part of gene was eliminated or inactivated by genetic engeneering) were completely rescued from developing peripartum cardiomyopathy by administering VEGF and Bromocriptine which provides strong evidence that cardiac angiogenic imbalance can lead to ultimate onset of peripartum cardiomyopathy.

  • Significant hemodynamic changes occur during pregnancy. Which can increase in preload secondary to the increase in red blood cell mass and blood volume.
  • Which increases the cardiac output by 20% to 30% due to an increase in heart rate and stroke volume by 15% to 25%.
  • All these changes present during the first and second trimester , it is not clear that hemodynamic stresses are the main reason for PPCM.[3]
  • Other etiologies such as myocarditis has been hypothesized due to the presence of viral genomes in biopsy of patients with PPCM as echo virus, Coxsackie, and parvovirus B19.
  • Genetic factors also have been implicated in the etiology of PPCM. Evidence of cluster of families with PPCM has been observed, and it is possible that the expression of the genes with the toxic environment during late pregnancy due to oxidative stress can increase the susceptibility of PPCM.
  • A pro-inflammatory state might play a role in the development of PPCM. Increased levels of cytokines such as TNF-alpha and interleukin-6 have been found in patients with PPCM and heart failure


Clinical Features

Pregnancy itself brings about some features that suggest cardiac insufficiency. Symptoms such as dyspnoea, dizziness, orthopnoea and decreased exercise tolerance are often normal findings in pregnancy.

Moreover, the normal cardiac physiology changes dramatically in the gravid female. Blood volume increases progressively from 6-8 weeks gestation (pregnancy) and reaches a maximum at approximately 32-34 weeks with little change thereafter. During the first trimester cardiac output is 30-40% higher than in the non-pregnant state; there is also an approximately 35% increase in stroke volume, a 15% increase in HR and a steady decrease in vascular resistance.

Since this is an excellent cover for a developing condition, often there are no extreme or notable symptoms until several days post-partum when the most notable symptoms become pulmonary edema with resultant breathing difficulties(dysnopea), serious energy depletion performing simple tasks such as walking, standing or even sitting up for extended periods, and sudden cardiac arrest.

If these conditions appear after a woman has been discharged from clinical care the possibility of mortality is greatly increased.

The dyspnoea is described usually by women as the inability to take a deep breath to get enough air into her lungs. It is thought that the hormonally mediated (progesterone) hyperventilation seen in pregnancy is the cause.

In PPCM the symptoms secondary to acute onset of heart failure seen are similar to patients with systolic dysfunction who are not pregnant. These symptoms include cough, orthopnoea, paroxysmal nocturnal dyspnoea, fatigue, palpitations, haemoptysis and chest pain. These symptoms mimic many normal pregnant women who cannot tolerate lying flat, have significant pedal edema, complain of shortness of breath and dyspnea but have no heart disease.

The challenge facing physicians is to differentiate between these two groups and diagnose women with PPCM sooner than later as early intervention has shown benefit and may save lives.

The following is a screening tool developed by James D. Fett, MD, which may be useful to patients and medical professionals in determining the need to take further action to diagnose symptoms:[4]

Focused medical history for PPCM screening, looking for early symptoms of heart failure, during last month of pregnancy:
1. Orthopnea (difficulty breathing while lying flat):
a.) None = 0 points; b.) Need to elevate head = 1 point; c.) Need to elevate upper body 45° or more = 2 points
2. Dyspnea (shortness of breath on exertion):
a.) None = 0 points; b.) Climbing 8 or more steps = 1 point; c.) Walking on level = 2 points
3. Unexplained cough:
a.) None = 0 points; b.) Night-time = 1 point; c.) Day and night = 2 points
4. Swelling (pitting edema) lower extremities:
a.) None = 0 points; b.) Below knee = 1 point; c.) Above and below knee and/or hands/face = 2 points.
5. Excessive weight gain during last month of pregnancy:
a.) Under 2 pounds per week = 0 points; b.) 2 to 4 pounds per week = 1 point; c.) over 4 pounds per week = 2 points.
6. Palpitations (sensation of irregular heart beats):
a.) None = 0 points; b.) When lying down at night = 1 point; c.) Day and night, any position = 2 points
Scoring and Action:
0 - 2 = low risk—continue observation
3 - 4 = mild risk—consider doing blood BNP and CRP; echocardiogram if BNP and CRP are elevated
5 or more = high risk—do blood BNP, CRP, and echocardiogram

Differentiating [disease name] from other Diseases

  • [Disease name] must be differentiated from other diseases that cause [clinical feature 1], [clinical feature 2], and [clinical feature 3], such as:
  • [Differential dx1]
  • [Differential dx2]
  • [Differential dx3]

Epidemiology and Demographics

  • The prevalence of [disease name] is approximately [number or range] per 100,000 individuals worldwide.
  • In [year], the incidence of [disease name] was estimated to be [number or range] cases per 100,000 individuals in [location].

Age

  • Patients of all age groups may develop [disease name].
  • [Disease name] is more commonly observed among patients aged [age range] years old.
  • [Disease name] is more commonly observed among [elderly patients/young patients/children].

Gender

  • [Disease name] affects men and women equally.
  • [Gender 1] are more commonly affected with [disease name] than [gender 2].
  • The [gender 1] to [Gender 2] ratio is approximately [number > 1] to 1.

Race

  • There is no racial predilection for [disease name].
  • [Disease name] usually affects individuals of the [race 1] race.
  • [Race 2] individuals are less likely to develop [disease name].


The reported incidence fluctuates globally but is higher in developing countries. It usually affects 1 in 300 to 1 in 3000 pregnancies. Nigeria(1%) and Haiti(0.3%) are examples of geographic hot spots of high incidence. In the Unites States, the incidence ranges between 1 in 3000 to 1 in 4000 deliveries.[5]


Risk Factors

  • Common risk factors in the development of [disease name] are [risk factor 1], [risk factor 2], [risk factor 3], and [risk factor 4].

The incidence of peripartum cardiomyopathy is increased in women over the age of 30, in twin pregnancies, in multiparous women, in women with gestational hypertension, those who have received tocolytic therapy, in maternal cocaine abuse and in african americans.

Natural History, Complications and Prognosis

  • The majority of patients with [disease name] remain asymptomatic for [duration/years].
  • Early clinical features include [manifestation 1], [manifestation 2], and [manifestation 3].
  • If left untreated, [#%] of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
  • Common complications of [disease name] include [complication 1], [complication 2], and [complication 3].
  • Prognosis is generally [excellent/good/poor], and the [1/5/10­year mortality/survival rate] of patients with [disease name] is approximately [#%].

Mortality rates range anywhere between 9% and 56%. About 50 to 60 percent of women with PPCM demonstrate improvement or total recovery in their left ventricular function within 6 months of diagnosis. The remainder tend to have either stabilization of their ventricular function or worsening (requiring cardiac transplantation).[6]

Women are strongly discouraged from subsequent pregnancies as numerous studies have demonstrated recurrence of the disease.

The New York Peripartum Cardiomyopathy Study Group is an ongoing registry seeking to answer many of the questions left unanswered because of the rarity of the disease. For more information about the study, one can visit [7]

Treatment for this disease varies widely reflecting the range of severity. While some patients with severe left ventricular dysfunction (less than 35%) may proceed to cardiac tranplant or require an automated internal cardiac defibrillator (AICD) and standard heart failure therapy, others may return to normal cardiac function and reuqire little, if any, additional medical therapy. However, all women should be strongly discouraged from having subsequent pregnancies.

Diagnosis

Diagnostic Criteria

  • The diagnosis of [disease name] is made when at least [number] of the following [number] diagnostic criteria are met:
  • [criterion 1]
  • [criterion 2]
  • [criterion 3]
  • [criterion 4]

Symptoms

  • [Disease name] is usually asymptomatic.
  • Symptoms of [disease name] may include the following:
  • [symptom 1]
  • [symptom 2]
  • [symptom 3]
  • [symptom 4]
  • [symptom 5]
  • [symptom 6]

Physical Examination

  • Patients with [disease name] usually appear [general appearance].
  • Physical examination may be remarkable for:
  • [finding 1]
  • [finding 2]
  • [finding 3]
  • [finding 4]
  • [finding 5]
  • [finding 6]

Laboratory Findings

  • There are no specific laboratory findings associated with [disease name].
  • A [positive/negative] [test name] is diagnostic of [disease name].
  • An [elevated/reduced] concentration of [serum/blood/urinary/CSF/other] [lab test] is diagnostic of [disease name].
  • Other laboratory findings consistent with the diagnosis of [disease name] include [abnormal test 1], [abnormal test 2], and [abnormal test 3].

Imaging Findings

  • There are no [imaging study] findings associated with [disease name].
  • [Imaging study 1] is the imaging modality of choice for [disease name].
  • On [imaging study 1], [disease name] is characterized by [finding 1], [finding 2], and [finding 3].
  • [Imaging study 2] may demonstrate [finding 1], [finding 2], and [finding 3].

Other Diagnostic Studies

  • [Disease name] may also be diagnosed using [diagnostic study name].
  • Findings on [diagnostic study name] include [finding 1], [finding 2], and [finding 3].


Echocardiography

Peripartum cardiomyopathy is diagnosed only when the following criteria are met: left ventricular ejection fraction (LVEF) <0.45 or M-mode fractional shortening <30% (or both) and end-diastolic dimension >2.7 cm/m².

Echocardiography is essential for diagnosis. It is effective and obviates the need for cardiac catheterization.[8]

It is also useful to diagnose mural thrombus, mitral regurgitation, tricuspid regurgitation and pericardial effusion.

Prompt echocardiography in all symptomatic pregnant patients can help to reveal patients with undiagnosed peripartum cardiomyopathy earlier in the course of the disease, thereby leading to earlier institution of care and to improvement of outcomes.

Treatment

Treatment for the disease is similar to treatment for congestive heart failure apart from concerning the adverse effect of treatment on fetus or breast-feeding infant.

Delivery is the recommended overall treatment to decrease the volume load, improve ventricular function and simplify the medical management of these patients.

  • The goal of treatment for patients with PPCM, optimizing the preload or volume status is done through appropriate diuresis and keeping a balance of intra- and extravascular volume.
  • Fluid restriction is fundamental to achieve this goal. Prepartum PCCM has special considerations for treatment due to side effects of medication that cross the placenta and affect the fetus. As an example, use of diuretics during pregnancy should be give in very low doses as they impair perfusion of the placenta and cause potential harm to the fetus. Both hydrochlorothiazide and furosemide are safe during pregnancy and lactation, with close monitoring for diuresis and at low doses.
  • Angiotensin-converting enzyme (ACE) inhibitors and angiotensin II receptor blockers (ARBs) are contraindicated during pregnancy due to the well known teratogenic effects if administrated during pregnancy.
  • Beta-blockers can be used with caution during pregnancy (beta-1 selective agents are preferred) and are contraindicated during breastfeeding because this is excreted in the breastmilk. Carvedilol is a combined beta-blocker with an additional alpha-blockade effect that allows a decrease of the afterload and is effective in the treatment of PPCM.
  • Hydralazine, a vasodilator, is safe during pregnancy. A nitroglycerin drip can be used to manage afterload in the acute setting. Nitroprusside is contraindicated during pregnancy for concern of cyanide's toxicity.
  • In very ill patients with hemodynamic instability, the use of inotropes might be necessary. Use of inotropes such as dobutamine, dopamine, and milrinone is restricted for this critical situation with close monitoring and with fast weaning off medication if possible.
  • Digoxin is another drug that can be used for the treatment of PPCM. It is safe during pregnancy and can be used when the ionotropic and chronotropic effect is necessary, especially in the uncontrolled atrial fibrillation.
  • Anticoagulation therapy in patients with PPCM is controversial. As a general recommendation, patients with PPCM without LV thrombus or atrial fibrillation should not be in anticoagulation. Patients with PPCM and atrial fibrillation and/or LV thrombus should be anticoagulated according to the guidelines for anticoagulation and the trimester of pregnancy.
  • Decisions regarding the use of an implantable cardioverter defibrillator (ICD) and cardiac resynchronization therapy in patients with PPCM should consider the natural history of these diseases, including the potential for the recovery of ventricular function.
  • Use of mechanical circulatory support has been describing in patients with fulminant PPCM. Placement of left ventricular assistant device (LVAD) can be a bridge for transplant or recovery.


Medical Therapy

  • There is no treatment for [disease name]; the mainstay of therapy is supportive care.
  • The mainstay of therapy for [disease name] is [medical therapy 1] and [medical therapy 2].
  • [Medical therapy 1] acts by [mechanism of action 1].
  • Response to [medical therapy 1] can be monitored with [test/physical finding/imaging] every [frequency/duration].

Surgery

  • Surgery is the mainstay of therapy for [disease name].
  • [Surgical procedure] in conjunction with [chemotherapy/radiation] is the most common approach to the treatment of [disease name].
  • [Surgical procedure] can only be performed for patients with [disease stage] [disease name].

Prevention

  • There are no primary preventive measures available for [disease name].
  • Effective measures for the primary prevention of [disease name] include [measure1], [measure2], and [measure3].
  • Once diagnosed and successfully treated, patients

References

  1. Shaikh N (2010). "An obstetric emergency called peripartum cardiomyopathy!". Journal of Emergencies, Trauma, and Shock. 3 (1): 39–42. doi:10.4103/0974-2700.58664. PMC 2823141. PMID 20165720. Retrieved 2012-06-05. Unknown parameter |month= ignored (help)
  2. Patten IS, Rana S, Shahul S, Rowe GC, Jang C, Liu L, Hacker MR, Rhee JS, Mitchell J, Mahmood F, Hess P, Farrell C, Koulisis N, Khankin EV, Burke SD, Tudorache I, Bauersachs J, del Monte F, Hilfiker-Kleiner D, Karumanchi SA, Arany Z (2012). "Cardiac angiogenic imbalance leads to peripartum cardiomyopathy". Nature. 485 (7398): 333–8. doi:10.1038/nature11040. PMID 22596155. Retrieved 2012-06-04. Unknown parameter |month= ignored (help)
  3. Rodriguez Ziccardi M, Siddique MS. PMID 29489231. Missing or empty |title= (help)
  4. Fett JD (2011). "Validation of a self-test for early diagnosis of heart failure in peripartum cardiomyopathy". Critical Pathways in Cardiology. 10 (1): 44–5. doi:10.1097/HPC.0b013e31820b887b. PMID 21562375. Retrieved 2012-06-05. Unknown parameter |month= ignored (help)
  5. Pearson GD, Veille JC, Rahimtoola S, Hsia J, Oakley CM, Hosenpud JD, Ansari A, Baughman KL (2000). "Peripartum cardiomyopathy: National Heart, Lung, and Blood Institute and Office of Rare Diseases (National Institutes of Health) workshop recommendations and review". JAMA : the Journal of the American Medical Association. 283 (9): 1183–8. PMID 10703781. Retrieved 2012-06-05. Unknown parameter |month= ignored (help)
  6. Ravikishore AG, Kaul UA, Sethi KK, Khalilullah M (1991). "Peripartum cardiomyopathy: prognostic variables at initial evaluation". International Journal of Cardiology. 32 (3): 377–80. PMID 1838741. Unknown parameter |month= ignored (help); |access-date= requires |url= (help)
  7. http://amothersheart.org/PRiCELESS.htm
  8. Bhakta P, Biswas BK, Banerjee B (2007). "Peripartum cardiomyopathy: review of the literature". Yonsei Medical Journal. 48 (5): 731–47. doi:10.3349/ymj.2007.48.5.731. PMC 2628138. PMID 17963329. Retrieved 2012-06-05. Unknown parameter |month= ignored (help)

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