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Multiple left ventricular inferoseptal clefts

Multimodality imaging appearance and differential diagnosis

Multiple linksventrikuläre inferoseptale Clefts

Darstellung mit multimodaler Bildgebung und Differenzialdiagnose

  • Case study
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Abstract

Background

Left ventricular inferoseptal clefts are a localized variant of myocardial structure, easily overlooked but potentially raising concern when identified through imaging.

Case study

Here we illustrate and describe inferoseptal clefts by means of multimodality imaging and consider them in relation to possible differential diagnoses. A 49-year-old male patient was investigated for chest pain and found to have multiple inferoseptal clefts. The pain subsequently resolved and was thought to have been pleuritic. There was no evidence or family history of hypertrophic cardiomyopathy. The diagnosis of clefts was arrived at after consultation with several cardiac imaging specialists and the few available relevant published reports. Echocardiography, cardiovascular magnetic resonance, invasive ventriculography and computed tomography each showed the clefts in relation to surrounding compact and contractile myocardium of the inferoseptal region, which occluded the clefts in systole. In terms of location, orientation and systolic occlusion the inferoseptal clefts resembled the isolated clefts reported in healthy volunteers, and have features in common with crypts reported in carriers of a genetic mutation associated with hypertrophic cardiomyopathy (HCM). The incidence and implications of multiple inferoseptal clefts have yet to be determined.

Conclusion

Multimodality imaging permits clear depiction of left ventricular inferoseptal clefts, which should be distinguished from different entities such as left ventricular noncompaction cardiomyopathy (LVNC), cardiac diverticula and cardiac aneurysms. Inferoseptal clefts have yet to be widely recognized as a distinct variant of regional left ventricular structure.

Zusammenfassung

Hintergrund

Multiple inferoseptale linksventrikuläre Clefts sind seltene myokardiale Strukturveränderungen. Die Kenntnis dieser Strukturvariante ist Voraussetzung einer präzisen differenzialdiagnostischen Abgrenzung gegenüber der linksventrikulären Noncompaction-Kardiomyopathie (LVNC) sowie gegenüber linksventrikulären Divertikeln und Aneurysmen. In der begrenzt verfügbaren Literatur wurden bislang einzelne oder paarige Clefts beschrieben.

Fallbericht

Berichtet wird über einen Fall eines 49-jährigen männlichen Patienten, der sich aufgrund akuter Thoraxschmerzen bei Vorliegen koronarer Risikofaktoren einer Herzkatheteruntersuchung unterzog mit dem Nachweis multipler inferoseptaler myokardialer Strukturveränderungen sowie einer Koronarsklerose. Die initiale Vermutung einer atypischen LVNC wurde nach Einsatz multimodaler Bildgebung mit Kontrastmittelechokardiographie (KM-Echo), Herzmagnetresonanztomographie (Kardio-MRT) sowie Herzcomputertomographie (Kardio-CT), Konsultation mehrerer Experten sowie Studium der Literatur revidiert zugunsten der Diagnose multipler inferoseptaler Clefts. Die Beschwerden des Patienten stellten sich im Verlauf als pleuritischer Genese dar. Inferoseptale Clefts sind diastolische myokardiale Spaltbildungen der linksventrikulären myokardialen Junktionsregion, die mittels einer schmalen myokardialen Schicht von dem rechten Ventrikel abgegrenzt sind. Die betroffene inferoseptale myokardiale Region zeigt im Bereich der zwischenliegenden myokardialen Bänder ein systolisches Kontraktionsverhalten mit Okklusion der Clefts und ähnelt den in der Literatur als Crypts beschriebenen Veränderungen, die bei Trägern einer genetischen HCM-Mutation (hypertrophische Kardiomyopathie) nachgewiesen wurden. Clefts wurden in einer Kardio-MRT-Beobachtung jedoch auch sowohl bei Gesunden wie auch bei einer Population unterschiedlicher Krankheitsentitäten (HCM, arterielle Hypertonie, Zustand nach operativer Therapie bei Fallot-Tetralogie bzw. Pulmonalklappenstenose) beschrieben.

Schlussfolgerung

Multimodales Imaging ermöglicht die präzise Diagnose multipler inferoseptaler Clefts. Zur besseren Einschätzung wird ein Überblick über die Literatur und die Differenzialdiagnose präsentiert. Bei genauer Kenntnis dieser myokardialen Strukturvariante kann eine kardiale Bildgebung ohne Strahlenbelastung (KM-Echo, Kardio-MRT) zur Diagnose ausreichend sein. Eine valide Diagnose ist erforderlich, um möglicherweise unnötige weitere diagnostische und therapeutische Schritte (Herzkatheteruntersuchung, Antikoagulation) zu vermeiden. Die Prävalenz dieser Strukturvariante sowie die Inzidenz klinischer Komplikationen sollten Gegenstand weiterer Untersuchungen bleiben.

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Acknowledgement

The authors would like to thank Professor Raimund Erbel, MD (University of Essen, Germany) and Professor Waldemar Hort, MD (University of Duesseldorf, Germany) for their helpful advice and discussions.

Conflict of interest

The authors declare that there are no conflicts of interest.

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Authors and Affiliations

  1. Department of Cardiology, Augusta-Krankenhaus, Amalienstr. 9, 40472, Duesseldorf, Germany

    M. Wein MD, A. Wolf-Puetz, R. Niehues, T. Klein & R. M. Klein

  2. University of Witten-Herdecke, Witten-Herdecke, Germany

    R. M. Klein

  3. Royal Brompton Hospital, London, UK

    P. J. Kilner

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  1. M. Wein MD
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  2. A. Wolf-Puetz
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  3. R. Niehues
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  4. T. Klein
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  6. R. M. Klein
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Correspondence to M. Wein MD.

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Wein, M., Wolf-Puetz, A., Niehues, R. et al. Multiple left ventricular inferoseptal clefts. Herz 36, 438–443 (2011). https://doi.org/10.1007/s00059-011-3424-9

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