Korean Circ J.  2010 Jul;40(7):339-342. 10.4070/kcj.2010.40.7.339.

The Management of Cardiovascular Abnormalities in Patient With LEOPARD Syndrome

• Lee KJ
• Choi H
• Choi WH
• Kwon SU
• Doh JH
• Namgung J
• Lee SY
• Lee WR

• KMID: 2225178
• DOI: http://doi.org/10.4070/kcj.2010.40.7.339

Abstract

LEOPARD syndrome (LS) is a rare hereditary disorder in Asian countries. This syndrome consists of multiple systemic abnormalities. In particular, characteristic cardiovascular effects in LS may include variable clinical manifestations from benign to life-threatening courses. The cardiac effects of this syndrome consist of left ventricular hypertrophy (LVH), pulmonary stenosis (PS), coronary artery dilatation and electrocardiogram(ECG) abnormalities. Since there are few LS patients who have undergone a complete cardiovascular evaluation, the nature and clinical prognosis of cardiovascular abnormalities in this syndrome remain uncertain. Also, there have been few reports on therapeutic strategies for cardiovascular abnormalities in LS. Here we describe a case of LS who presented with multiple cardiovascular problems and underwent successful surgical and medical treatment.

Keyword

LEOPARD syndrome; Lentigo; Pulmonary valve stenosis

MeSH Terms

Asian Continental Ancestry Group
Cardiovascular Abnormalities
Coronary Vessels
Dilatation
Humans
Hypertrophy, Left Ventricular
Lentigo
LEOPARD Syndrome
Panthera
Prognosis
Pulmonary Valve Stenosis

Figure

• 

  Fig. 1 Typical ocular hypertelorism is seen on the patient's face (A). Lentigines, of large dermographic black-brownish character, are distributed over his arm, chest (B and C). The initial ECG shows ventricular tachycardia that originated from the apical inferoseptal left ventricular wall (D). Follow-up ECG after intravenous amiodarone injection demonstrates normal sinus rhythm with right bundle branch block (E). ECG: electrocardiogram.

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  Fig. 2 Initial echocardiography showing diffuse left ventricular hypertrophy (A). Initial pulmonic valve peak pressure gradient and right ventricular systolic pressure by use of continuous wave Doppler are, respectively, 69.6 mmHg (B) and 81.9 mmHg (C). Cardiac 64-MDCT demonstrating post-stenotic dilatation of the pulmonary trunk (arrows) (D) with a maximal diameter of 45.4 mm.

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  Fig. 3 Follow-up pulmonic valve peak pressure gradient and right ventricular systolic pressure by use of continuous wave Doppler are, respectively, 10.1 mmHg (A) and 38.1 mmHg (B). Follow-up Cardiac 64-MDCT demonstrating a marked reduction in post-stenotic dilatation of the pulmonary trunk, with a maximal diameter of 37.6 mm (arrows) (C).

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