Assessment of Right Heart Functions in Children with Mild Cystic Fibrosis

ABSTRACT

Background Cystic fibrosis (CF) is a multisystemic disease that prevalently involves the lungs. Hypoxemia occurs due to the existing of progressive damage to the pulmonary parenchyma and pulmonary vessels. The condition may cause systolic and diastolic dysfunction to the right ventricle due to the effects of high pulmonary artery systolic pressure (PASP). The study aimed to determine echocardiographic alterations in PASP, right ventricle (RV) anatomy, and functions in mild CF children.

Materials and Methods RV anatomy, systolic, and diastolic functions were evaluated with conventional echocardiographic measurements. Estimated PASP was used measured with new echocardiographic modalities, including pulmonary artery acceleration time (PAAT), right ventricular ejection time (RVET), and their ratio (PAAT/RVET). The obtained echocardiographic data were statistically compared between the patient group and the control group.

Results The study consisted of 30 pediatric patients with mild CF and 30 healthy children with similar demographics. In patient group, conventional parameters disclosed differences in RV anatomy, both systolic and diastolic functions of RV compared with the healthy group. We did not compare the patient group with published standard data because of the wide range variability. However, new echocardiographic parameters showed notable increase in pulmonary artery pressure compared with values of control group and published standard data (p<0.001).

Conclusion Elevated PASP, RV failure, and Cor pulmonale usually begin early in children with mild CF. In addition to routine echocardiographic measurements to evaluate RV, we recommend the use of new echocardiographic modalities for routine examinations and in the follow up of children with mild CF.

ZUSAMMENFASSUNG

Hintergrund und Ziel Die Mukoviszidose ist eine multisystemische Erkrankung, bei der die Lungenbeteiligung im Vordergrund steht. Die fortschreitende Schädigung des Lungenparenchyms und der pulmonalen Gefäße führt letztlich zur Hypoxämie. Durch den hohen systolischen Pulmonalarteriendruck (PASP) kann eine systolische und diastolische Dysfunktion des rechten Ventrikels bei Mukoviszidosepatienten auftreten. Ziel der Studie war es, die echokardiographischen Veränderungen des PASP sowie der rechtsventrikulären Anatomie und Funktion bei Kinder mit leichter Mukoviszidose zu bestimmen.

Patienten und Methodik Die Beurteilung der Anatomie sowie der systolischen und diastolischen Funktionen des rechten Ventrikels erfolgte mittels konventioneller echokardiographischer Messungen. Zur Anwendung kann der abgeschätzte PASP, der mittels neuer echokardiographischer Parameter, darunter die pulmonalarterielle Akzelerationszeit (PAAT, Pulmonary Artery Acceleration Time), die rechtsventrikuläre Ejektionszeit (RVET, Right Ventricular Ejection Time) und deren Verhältnis (PAAT/RVET), ermittelt wurde. Anhand der so erhobenen echokardiographischen Daten erfolgte dann ein statistischer Vergleich von Patientengruppe und Kontrollgruppe.

Ergebnisse Insgesamt wurden 30 pädiatrische Patienten mit leichter Mukoviszidose und 30 gesunden Kinder mit ähnlichen demographischen Daten in die Studie aufgenommen. In der Patientengruppe fanden sich bei Betrachtung der üblichen Parameter Unterschiede in der Anatomie sowie der systolischen und diastolische Funktion des rechten Ventrikels gegenüber der Gruppe der gesunden Kinder. Angesichts der erheblichen Variabilität verzichteten wir auf einen Vergleich der Patientengruppendaten mit den veröffentlichten Standarddaten. Hingegen zeigten die neuen echokardiographischen Parameter einen signifikanten Anstieg des Pulmonalarteriendrucks im Vergleich zu den Werten der Kontrollgruppe und den veröffentlichten Standarddaten (p<0.001).

Schlussfolgerung Bei Kindern mit leichter Mukoviszidose finden sich PASP-Erhöhung, Rechtsherzinsuffizienz und Cor pulmonale in der Regel schon früh im Krankheitsverlauf. Wir empfehlen, zusätzlich zur Messung der echokardiographischen Routineparameter zur Beurteilung des rechten Ventrikels neuere echokardiographische Modalitäten bei den Routine- und Kontrolluntersuchungen von Kindern mit leichter Mukoviszidose zu verwenden.

Keywords

Cystic fibrosis - Pulmonary hypertension - Pulmonary function test - Cor Pulmonale - Childhood Age Group

Schlüsselwörter

Mukoviszidose - Pulmonale Hypertonie - Lungenfunktionstest - Cor Pulmonale - Altersgruppe der Kinder

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