Peripartum dilated cardiomyopathy


Dr. José Joaquín Martínez, Intensivist Cardiologist Internist. Intensive Cardiovascular Care Unit of the General Hospital of the Plaza de la Salud.

It is a rare heart disease that is characterized because the heart dilates (increases in size) and there are symptoms of heart failure such as dyspnea, edema of the lower limbs and fatigue, which are related to physical activity at first, but which can progress and worsen until appearing at rest making the daily activities difficult for the patient.

It occurs at the end of pregnancy or the first five months after childbirth (puerperium) in a mother who had no heart disease at pregnancy.

The first description of the disease is attributed to Ritchie in 1849.

The European Society of Cardiology defines it as an unfamiliar, non-genetic form of cardiomyopathy associated with pregnancy. The American Heart Association, as a rare form of primary, acquired cardiomyopathy associated with left ventricular failure, in the absence of a cause of primary recognizable heart failure. This demonstrable left ventricular systolic dysfunction, by classical echocardiographic criteria.

The incidence of this heart disease worldwide is very variable, with areas where it is a rarity and other places, such as Haiti, where the disease is quite frequent. It is necessary for its diagnosis that there is no other probable cause of the cardiac involvement, that is to say that the mother who suffers it, a hypertensive, rheumatic, or ischemic heart disease.

In this sense, the diagnosis of peripartum dilated cardiomyopathy is a diagnosis of exclusion. Its etiology (cause) is not well known, and its frequency is variable, there are countries where it is rare and others where it is a very important cause of heart failure and, consequently, death.

The first description of this disease was made in 1870 and it was not until 1971 when the term peripartum dilated cardiomyopathy was coined. It is a very rare disease in industrialized countries and relatively frequent in developing countries.

In the United States, one case is recorded for every 2300 live births, one for every 1000 in South Africa, one for every 300 in Haiti, and one for every 100 in Nigeria.

Etiology and epidemiology

Its causes are not known with certainty, but it is known that there are factors that increase the risk of suffering from it, such as multiparity (more than one birth or having a twin pregnancy, for example); maternal age (more common in mothers over 30 years old); race (women of African descent seem more prone to it); suffering from pregnancy-induced hypertension (preclamsia), having had viral infections during pregnancy (more than one birth) among others.

Also having suffered this condition in previous pregnancies increases the risk of having it in subsequent pregnancies.

Within the mechanisms involved in this disease there is talk of an inflammatory theory, with the participation of the immune system, a viral theory that involves the participation of certain viral infections in the course of pregnancy and there is a hormonal theory, in which substances such as Estrogen and prolactins among others.

Clinical manifestations 

The precise mechanism that produces this cardiomyopathy is unknown, but there are a large number of factors involved: hypertension, diabetes, smoking, mother's age, etc.

At first the symptoms can be very subtle and even go unnoticed, as fatigue is mild and edema of the lower limbs, (maleolar) as well as dyspnea (respiratory distress) can usually be seen in the final stage of pregnancy, so Many pregnant women do not give due importance to this symptomatology.

These symptoms usually increase by increasing respiratory distress, edema, and knotted to this a lot of functional disability arises for the mother.

You may have palpitations due to the occurrence of cardiac arrhythmias, and as the disease progresses, the functional disability of the patient increases, resulting in fatigue and dyspnea at rest.

The edema can increase extending from the feet to the abdomen with the appearance of ascites (swelling of the abdomen due to the presence of fluid in the peritoneal cavity).

In the very advanced stage of the disease, the so-called paroxysmal nocturnal dyspnea may occur, which consists in the impossibility of lying on the bed in a flat position (supine position), so it is forced to get out of bed to breathe.

This last picture can be repeated until a life-threatening complication appears, such as acute lung edema, which represents an emergency in cardiology that often requires the use of assisted mechanical ventilation.

Diagnosis and treatment

The diagnosis of peripartum cardiomyopathy is sometimes complicated by the non-specificity and subtlety of the symptoms at first. There is no specific clinical criterion that is pathognomonic (proper) of this entity.

Its diagnosis is based on a high clinical suspicion, together with the time of onset of symptoms (end of pregnancy or five months after delivery) that should then be corroborated with paraclinical studies, such as chest x-rays, electrocardiogram and an echocardiogram that It is basic and cornerstone to support the diagnosis.

Without the echocardiographic data of an ejection fraction less than 45 % and a shortening fraction less than 30%, more dilatation of the left ventricle, greater than 2.7 cm / m2 of body surface area, the diagnosis cannot be sustained.

The key to making the diagnosis is to always be on the lookout for any suspicious symptoms, to initiate site studies.

Once the diagnosis is made, it is implemented in treatment, which in the case that the woman is pregnant does not differ from the treatment established for a heart failure of another nature.

The problem arises when the disease occurs during pregnancy, as some of the drugs used are contraindicated by putting the health of the fetus at risk.

In general, there is a non-pharmacological treatment and a pharmacological treatment.


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