Echokardiografische Diagnose des aortopulmonalen Fensters

Zusammenfassung

Ziel/Material und Methoden:Das aortopulmonale Fenster ist eine sehr seltene angeborene kardiovaskuläre Malformation mit Verbindung zwischen Aorta ascendens und dem Truncus pulmonalis. Wir beschreiben die charakteristischen echokardiografischen Befunde und die Grenzen der nichtinvasiven Diagnostik anhand von 5 Patienten mit diesem Krankheitsbild, die in unserer Abteilung im Zeitraum von 7/2000 bis 11/2005 betreut wurden. Ergebnisse: Wegweisend für die echokardiografische Diagnose war bei allen Kindern der Nachweis einer erheblichen Volumenbelastung des linken Vorhofes und Ventrikels. In allen Fällen konnte das Fenster echokardiografisch direkt dargestellt werden. Dopplersonografisch fanden sich deutliche Zeichen eines Windkessellecks mit diastolisch negativem Fluss in der Aorta descendens und in Systemarterien. Alle 5 Patienten wiesen zusätzliche Herzfehler auf, welche sämtlich echokardiografisch diagnostiziert wurden. Koronaranomalien konnten bei allen Patienten nichtinvasiv ausgeschlossen werden. Schlussfolgerung: Die Echokardiografie und Farbdoppler-Sonografie ermöglicht die zuverlässige Diagnose des aortopulmonalen Fensters. Voraussetzung ist die systematische Darstellung aller kardialen Details, da ein aortopulmonales Fenster meist in Kombination mit gravierenden weiteren kardialen Anomalien auftritt, die häufig bereits geeignet wären, die abnorme Hämodynamik des Patienten zu erklären. Eine invasive Herzkatheteruntersuchung wird nur erforderlich, wenn Koronaranomalien echokardiografisch nicht zweifelsfrei ausgeschlossen werden können oder Hinweise auf eine potenziell fixierte pulmonale Hypertonie vorliegen.

Abstract

Purpose/Materials and Methods: The aorto-pulmonary window is a rare congenital cardiac malformation consisting of a connection between the ascending aorta and the main pulmonary artery. Based on 5 consecutive patients who were treated in our tertiary referral centre between 7/2000 and 11/2005, we report the echocardiographic features as well the limitations of this diagnostic method in the assessment of this rare malformation. Results: Dilation of the left atrium and ventricle due to the large left-to-right-shunt was the characteristic echocardiographic feature in all patients with aorto-pulmonary window. In all patients we were able to visualise the window by 2D-echocardiography. Pulsed- and continous-wave Doppler showed diastolic negative flow in the descending aorta and in systemic arteries due to left-to-right shunting in diastole via the aorto-pulmonary window. All 5 patients had significant associated cardiovascular malformations. They were accurately diagnosed by echocardiography. Coronary anomalies could be excluded noninvasively in all patients. Conclusion: Echocardiography is the method of choice for diagnosis of aortopulmonary window. Detection of aortopulmonary window, however, requires a systematic and careful investigation of all cardiovascular anatomic details, since this anomaly is frequently associated with substantial additional cardiac anomalies, which would by themselves be sufficient to explain the haemodynamic abnormalities of the patient. Cardiac catheterisation is required only in cases with inadequate information about coronary artery anatomy or in older patients with pulmonary hypertension, to determine the patient’s suitability for surgical correction.

Key words

aortopulmonary window - echocardiography - congenital heart disease

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