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Isolierte kongenitale Koronaranomalien

Morphologie und klinische Relevanz im Kindes- und Jugendalter

Isolated congenital anomalies of the coronary arteries

Morphology and clinical implications in childhood and adolescence

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Zusammenfassung

Hintergrund

Isolierte Koronaranomalien sind seltene angeborene kardiovaskuläre Fehlbildungen. Zu ihnen zählen der Fehlabgang einer Koronararterie aus der Pulmonalarterie (ALCAPA, ARCAPA), der atypische Abgang aus einem nicht zugehörigen Aortensinus, Koronarfisteln und Koronaraneurysmen.

Diagnostik

Die Klinik variiert von akut lebensbedrohlichen Symptomen wie Myokardinfarkt, kardialer Synkope und ventrikulären Arrhythmien über die Volumenbelastung einzelner Herzhöhlen bis hin zu asymptomatischen Verläufen. Diagnostisch steht die Echokardiographie als Screeningmethode zur Verfügung; zur genauen morphologischen Darstellung der Gefäßsituation kommen bildgebende Verfahren wie Magnetresonanztomographie oder Computertomographie und als Goldstandard die Koronarangiographie zur Anwendung.

Therapie

Die Indikation zur konservativen, interventionellen oder operativen Behandlung ist jeweils individuell zu stellen.

Abstract

Background

Isolated coronary anomalies are rare congenital malformations. The anomalous origin of the coronary artery from the pulmonary artery (ALCAPA, ARCAPA), the anomalous origination of the coronary ostium from the opposite, facing “coronary” sinus, coronary fistulas, and coronary aneurysms have to be mentioned.

Diagnosis

The symptoms vary from life-threatening events (e.g., myocardial infarction, cardiac syncope and ventricular dysrhythmia) to atrial or ventricular volume overload and asymptomatic courses. Echocardiography is used as a screening method; noninvasive modalities such as magnetic resonance imaging and computed tomography are additionally used for diagnosis, but coronary angiography is the gold standard to depict the morphology discussed in the individual case.

Therapy

The indication for conservative, interventional, or operative treatment must be decided for each patient individually.

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Abbreviations

ALCA:

„Anomalous left coronary artery“

ALCAPA:

„Anomalous origin of the left coronary artery from the pulmonary artery“

ARCA:

„Anomalous right coronary artery“

ARCAPA:

„Anomalous origin of the right coronary artery from the pulmonary artery“

AV:

Atrioventrikulär

aVL:

EKG-Ableitung („augmented voltage left“)

aVR:

EKG-Ableitung („augmented voltage right“)

CA:

„Coronary aneurysm“

CALM:

„Congenital absence of the left main coronary artery“

CAF:

„Coronary artery fistula“

CCF:

Coronary-cameral-fistula

ccTGA:

„Congenitally corrected transposition of the great arteries“

CT:

Computertomographie

CVF:

„Coronary vascular fistula“

d-TGA:

Dextro-Transposition der großen Gefäße

EF:

Ejektionsfraktion

EKG:

Elektrokardiogramm

FS:

Verkürzungsfraktion

HK:

Herzkatheter

LAD:

R. interventricularis anterior

LCA:

„Left coronary artery“

LCx :

R. circumflexus

LPA:

„Left pulmonary artery“

MI:

Mitralinsuffizienz

MRT:

Magnetresonanztomographie

PET:

Positronenemissionstomographie

RCA:

Rechte Koronararterie

RPA:

„Right pulmonary artery“

SSW:

Schwangerschaftswoche

TGA:

Transposition der großen Gefäße

UKG:

Echokardiographie

VES :

Ventrikuläre Extrasystolen

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Authors and Affiliations

  1. Klinik für Kinderkardiologie und angeborene Herzfehler, Deutsches Herzzentrum München, Lazarettstraße 36, 80636, München, Deutschland

    M. Hauser, C. Meierhofer & H. Kaemmerer

  2. Franz-Schrank Straße 2, München, Deutschland

    M. Hauser

  3. Institut für Radiologie und Nuklearmedizin, Deutsches Herzzentrum München, München, Deutschland

    A. Will

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  1. M. Hauser
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  2. C. Meierhofer
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Correspondence to M. Hauser.

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Hauser, M., Meierhofer, C., Will, A. et al. Isolierte kongenitale Koronaranomalien. Monatsschr Kinderheilkd 161, 225–233 (2013). https://doi.org/10.1007/s00112-012-2813-3

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