06 November 2018

Today, most children with congenital heart disease survive to adulthood. However, these patients need lifelong treatment for their heart problems and often repeated cardiac surgeries. Aleksandra Trzebiatowska-Krzynska, PhD-student and cardiologist, researches better methods to predict when it's time for the next surgery, and thus try to reduce the number of operations a particular individual needs to undergo.

Aleksandra Trzebiatowska-Krzynska
Aleksandra Trzebiatowska-Krzynska Emma Busk Winquist
Adult patients with Congenital Heart Disease (GUCH) are now a relatively large patient group. At the Cardiological clinic at the University Hospital in Linköping there are approximately 900 GUCH patients registered and having regular checkups. Previously these patients had an increased risk of complications, but with improved surgical methods most people now expect a normal life span.

GUCH patients are a diverse patient group. They may suffer from one of several hundred different variants of heart disease, which contributes to the difficulty in researching them. In her study, Alexandra Trzebiatowska-Krzynska has focused on patients with Fallot's anomaly or transposition of the great arteries*. During life, most of them will need to undergo repeated surgical procedures to replace heart valves and correct other problems. Some artificial heart valves need to be replaced after a number of years, and being able to predict when could mean that operations are carried out at an optimal time and the number of operations that patients undergo during their lifetime would be fewer.

In an article recently published in the journal PlosOne (Afterload dependence of right ventricular myocardial deformation: A comparison between tetralogy of Fallot and atrially corrected transposition of the great arteries in adult patients), Aleksandra Trzebiatowska-Krzynska’s research group examines which measurements of cardiac function (eg "strain") may help predict when it is time for surgery. "Strain" is a measure of how the cardiac muscle wall contracts, the tension of the muscle itself. It can be measured for the whole heart, but also for smaller regions such as the right ventricle. The study showed that "strain" reflects early changes in the way the muscle works and pumps, which may alert the attending physician that the patient is in need of improved drug treatment or new surgery. More research is needed to verify the results of the study.

– "In Sweden there are several centers for GUCH patients with mutual guidelines for treatment. A large amount of data has already been collected - ECG, ultrasound, MR and so on. This data provides a basis for greater cooperation for the patients' wellbeing, says Aleksandra Trzebiatowska-Krzynska.

International studies on "strain" in connection with various heart diseases are ongoing. There are several major collaborations between different research groups, including the Netherlands, Canada and the United States.

*Children with Fallot's anomaly have a narrowing at the pulmonary valves, a hole in the partition between the heart chambers and a thickened right chamber wall. The restriction of bloodflow to the pulmonary artery coupled with the direct outflow of blood from the right ventricle results in some or all of the blood never reaching the lungs for oxygenation before it is pumped back into the body. In the case of transposition of the great arteries, the aorta and pulmonary artery have switched place, which creates two independent circuits in parallell instead of serially coupled. This prevents the oxygenated blood from reaching the body. Both of these conditions require immediate surgery early in life, often within hours after delivery,  if the child is to survive.
 

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