Wolff-Parkinson-White (WPW) Syndrome in Isolated Noncompaction of the Ventricular Myocardium (INVM)-Three Cases-

Abstract

solated noncompaction of the ventricular myocardium (INVM) is a rare disorder characterized by excessively prominent trabeculations and deep intertrabecular recesses, mainly in the left ventricle without other congenital cardiac malformations. 1 Most patients with INVM show various abnormalities on the electrocardiogram (ECG), including Wolff-Parkinson-White (WPW) syndrome, 2,3 which is currently classified into several types based on the localization of the bypass tract. It has not previously been reported which type of WPW syndrome occurs in patients with INVM. 3,4 We report 3 patients with INVM and WPW syndrome type B on ECG, which has not been previoulsy reported. 3,4 Case Reports Patient 1A 9-month-old Japanese girl was admitted to hospital because of increasing dyspnea. She was the first child of healthy nonconsanguineous parents, and the pregnancy and delivery had been uneventful.On admission, she was thin and malnourished: weight 6 kg and height 56 cm, both below the 10th percentile. She was pale and had cyanosis of the lips and nail bed. On auscultation, there was tachypnea and moderate retraction of the chest wall with moist rales over the entire lung field. A grade 3 pansystolic murmur was noted at the apex. Her abdomen was moderately distended, with the liver palpable 3 cm below the right costal margin. Chest X-ray showed marked cardiac enlargement, and pulmonary venous congestion. She died suddenly at 11 months of age. Patient 2The 6-month-old younger sister of Patient 1 was referred for examination of her cardiac function. The pregnancy and delivery had been uneventful and she was currently alert and healthy. Her heart sounds were clear and the rhythm was regular without murmur.Chest X-ray showed mild cardiac enlargement with a cardiothoracic ratio of 0.61. One year later, she still did not have symptoms, but her cardiac performance had deteriorated slightly, with a left ventricular fractional shortening (LVFS) of 24% and an ejection fraction (LVEF) of 57%. She was treated with oral angiotensin-converting enzyme inhibitor. Patient 3A 9-year-old girl was referred for an outpatient examination of an arrhythmia diagnosed previously during a school physical examination. She was alert and healthy with clear heart sounds and no murmur. No cardiac enlargement was noted on the chest roentgenogram. Electrocardiography, Echocardiography and Magnetic Resonance ImagingThe ECG of Patient 1 revealed marked tachycardia of 150 beats/min and WPW syndrome type B, with localization of the accessory pathway in the right anteroseptal area (Fig 1A). Cardiac performance was markedly depressed. A 2-dimensional (D) echocardiogram (Fig 1B) and a T1-weighted magnetic resonance imaging (MRI) scan (Fig 1C) revealed numerous, excessively prominent ventricular trabeculations and deep intertrabecular recesses, but there was no evidence of endocardial thrombus over the left ventricular wall.The ECG from Patient 2 revealed a normal sinus rhythm and WPW syndrome type B, with localization of the accessory pathway in the right anteros...

Left ventricular noncompaction cardiomyopathy: updated review

“…The frequency and type of arrhythmia may vary by age. Wolff-Parkinson-White (WPW) syndrome [Nihei et al 2004] and ventricular tachycardia are more commonly seen in children, while atrial fibrillation and ventricular arrhythmias were reported in 25% and 47% of adults with LVNC respectively [Ritter et al 1997]. Other rhythm abnormalities that have been reported in electrocardiogram (EKG) are paroxysmal supraventricular tachycardia, left or right bundle branch block, fascicular block, biventricular hypertrophy, T-wave inversions, AV block and complete heart block [Ichida et al 1999;Reynen et al 1997].…”

Left Ventricular Noncompaction

“…Typical patients with isolated LVNC have an ECG demonstrating biventricular hypertrophy with extreme QRS voltages, similar to those seen in infants with Pompe's disease, and isolated or diffuse T-wave changes. A high incidence of Wolff-Parkinson-White (WPW) has been reported in children, 8,35,36 to which arrest of the normal process of development in the annulus fibrosus can be attributed. 35 …”

“…A high incidence of Wolff-Parkinson-White (WPW) has been reported in children, 8,35,36 to which arrest of the normal process of development in the annulus fibrosus can be attributed. 35 …”

Left Ventricular Noncompaction and Pre-Excitation: An Unusual Finding in Adults

“…The association of WPW with LVNC is well recognized. Previous reports include WPW associated with isolated LVNC 3,7,8 ; WPW associated with LVNC and Fabry disease 9 or hypertrophic cardiomyopathy 10 was also reported. During embryogenesis there is direct continuity of the atrial and ventricular myocardium, which is ultimately disrupted as the annulus fibrosus develops.…”