Pitfalls in Echocardiography: Coarctation of the Aorta Presenting as Dilated Cardiomyopathy (DCM)

Abstract

Purpose: The morphologic spectrum of aortic coarctation extends from discrete isthmic obstruction to tubular hypoplasia of the entire aortic arch. Neonates with coarctation frequently present with congestive heart failure and critically reduced perfusion of the descending aorta following ductal closure. During the recent years we observed several infants with coarctation who presented beyond the neonatal period with dilated cardiomyopathy (DCM). We reviewed our patients with coarctation to determine whether this presentation represents an exception or is relevant for the differential diagnosis of children with DCM.

Materials and Methods: From 1/2001 to 12/2013 74 babies with isolated coarctation were diagnosed in our institution. 50 patients presented in the neonatal period and 24 patients beyond the first month.

Results: 5/74 infants presented after the neonatal period with poorly contractile, dilated left ventricles. Echocardiographic detection of the coarctation was facilitated by application of the ductal view and by Doppler interrogation of the celiac artery revealing a significantly diminished systolic flow velocity. All patients underwent resection of the coarctation and end-to-end anastomosis of the aorta. Postoperative normalization of left ventricular function was observed within a median interval of 2 months.

Conclusion: Coarctation of the aorta presenting as DCM accounted for 21 % of our infants with coarctation who presented beyond the neonatal period and 7 % of those in the first year of life. The stenosis was difficult to detect because of its distal location and normal configuration of the aortic arch. Isthmic coarctation should be included in the differential diagnosis of infants with DCM and requires careful examination of the isthmic region in these children.

Zusammenfassung

Ziel: Das morphologische Spektrum der Aortenisthmusstenose reicht von diskreter Obstruktion im Isthmusbereich bis hin zur Hypoplasie des gesamten Bogens. Neugeborene mit Aortenisthmusstenose fallen meistens durch Herzinsuffizienz und verminderte Perfusion der deszendierenden Aorta auf. Wir berichten über 5 Säuglinge mit Aortenisthmusstenose, die sich unter dem Bild einer dilatativen Kardiomyopathie (DCM) präsentierten.

Material und Methoden: Zwischen 1/2001 und 12/2013 wurden 74 Säuglinge mit isolierter Aortenisthmusstenose in unserer Klinik vorgestellt. 50 Patienten präsentierten sich in der Neonatalperiode, 24 Patienten nach den ersten 4 Lebenswochen.

Ergebnisse: 5/74 Säuglingen stellten sich nach der Neugeborenenperiode mit einem deutlich dilatierten und schlecht kontraktilen linken Ventrikel vor. Echokardiografisch erfolgte die Diagnosestellung mittels „ductal view“ sowie anhand eines deutlich reduzierten systolischen Dopplerflussprofils im Truncus coeliacus. Bei allen Patienten erfolgte die Resektion der Koarktation und End-zu-End-Anastomose der Aorta. Postoperativ zeigte sich eine Normalisierung der linksventrikulären Funktion nach einem medianen Zeitintervall von 2 Monaten.

Schlussfolgerung: Aortenisthmusstenosen, die sich als DCM präsentierten, betrafen 21 % aller Säuglinge über 4 Wochen, entsprechend 7 % aller Aortenisthmusstenosen, die im ersten Lebensjahr auffielen. Aufgrund der distalen Lokalisation der Aortenisthmusstenose und dem unauffälligen Aortenbogen ist die Stenose echokardiografisch schwer diagnostizierbar. Eine sorgfältige Untersuchung der Isthmusregion ist bei allen Patienten mit DCM zwingend erforderlich.

Key words

aortic coarctation - dilated cardiomyopathy - infant - congestive heart failure

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