Angiographic Demonstration of Intramyocardial Sinusoids in Pulmonary-Valve Atresia with Intact Ventricular Septum and Hypoplastic Right Ventricle

Lauer, Ronald M.; Fink, Howard P.; Petry, Eugene L.; Dunn, Marvin; Diehl, Antoni M.

doi:10.1056/nejm196407092710203

Cited by 128 publications

(49 citation statements)

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“…All 3 were diagnosed on the basis of the characteristic numerous, excessively prominent trabeculations and deep intertrabecular recesses on echocardiography. 1,4 Congenital cardiac anomalies that have sometimes been reported in the myocardial pattern of persistent sinusoids, such as ventricular outflow tract obstruction, 5,6 were not noted.…”

Section: Discussionmentioning

confidence: 99%

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

Nihei

Shinomiya

Kabayama

et al. 2004

Circ J

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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...

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Section: Discussionmentioning

confidence: 99%

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

Nihei

Shinomiya

Kabayama

et al. 2004

Circ J

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“…In the majority of the reports in the literature, noncompaction of the ventricular myocardium is associated with other congenital heart diseases, among which pulmonary atresia and obstruction of the left ventricular outflow tract combined with an intact interventricular septum predominate [1][2][3] . In these cases, noncompaction of the ventricular myocardium occurs because the ventricles are exposed to very high pressures during the intrauterine development.…”

Section: Discussionmentioning

confidence: 99%

“…Persistence of the noncompaction of the myocardium is a rare entity, usually diagnosed in the pediatric population and associated with other structural congenital malformations of the heart. It predominates in patients with congenital obstruction of the right or left ventricle outflow tract [1][2][3][4] . Isolated noncompaction of the myocardium, defined by absence of any other associated structural cardiac alteration, is an even rarer form of the entity, with only a few reports in the literature [5][6][7][8] .…”

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confidence: 99%

Isolated noncompaction of the myocardium

Elías¹,

Valadão²,

Kuniyoshi³

et al. 2000

Arq. Bras. Cardiol.

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We report the case of a 42-year-old female with fatigue on exertion and palpitation consequent to the existence of isolated noncompaction of the myocardium. We discuss clinical and familial findings, diagnostic possibilities, and prognostic and therapeutical implications of this rare disorder of endomyocardial morphogenesis.During the initial phase of embryonic development, the heart is a trabecular net with a spongy myocardium. The intertrabecular spaces communicate with the cardiac chambers. As the heart develops, the myocardium condenses and the intertrabecular recesses are reduced to capillaries. Trabecular compaction is usually more complete in the left ventricle than in the right ventricle. Noncompaction of the ventricular myocardium is defined as an anomaly of endomyocardial morphogenesis, and it is believed to be an arrest in the compaction of the myocardial fibers, which meet forming an interwoven loose net during intrauterine life. Persistence of the noncompaction of the myocardium is a rare entity, usually diagnosed in the pediatric population and associated with other structural congenital malformations of the heart. It predominates in patients with congenital obstruction of the right or left ventricle outflow tract [1][2][3][4] . Isolated noncompaction of the myocardium, defined by absence of any other associated structural cardiac alteration, is an even rarer form of the entity, with only a few reports in the literature [5][6][7][8] . Isolated noncompaction of the myocardium may be identified from childhood to adulthood. Its diagnosis may be delayed because of some findings resembling other clinical entities. Both sexes may be affected and familial recurrence may occur.We report the case of a 42-year-old female with isolated noncompaction of the myocardium, describing the clinical and familial history, diagnostic possibilities, and therapeutical approach. We also review the literature about this rare disorder of endomyocardial morphogenesis. Case reportA 42-year-old female complaining of fatigue on medium exertion, chest pain, and palpitation for 10 years was referred to our service to investigate arrhythmia. She reported loosing 2 children in the first year of life, one due to sudden death at the age of 5 months and the other due to refractory heart failure in the first week of life. The anatomicopathological study of the latter child performed in another service showed a dilated left ventricle with increased and abnormal trabeculation, whose final diagnosis, at the time, was cardiomyopathy of undetermined etiology. She also reported two siblings who had sudden death during childhood, and one uncle and one cousin, who died due to refractory congestive heart failure. The physical examination was normal. The 12-lead electrocardiogram showed sinus rhythm and deviation of the axis to the right in the frontal plane without meeting the criteria of left posteroinferior divisional heart block (figura 1A). Chest X-ray was normal. The exercise test showed depression of the ST segment associated with typical...

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“…Підви-щена трабекулярність звичайно не зустрічається у постнатальному періоді [4]. Найчастіше ІНЛШ спостерігається в поєднанні з різними вадами сер-ця [5]. Уперше кардіоміопатія як самостійна нозо-логічна форма описана Thomas K. Chin та співавт.…”

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Некомпактна Кардіоміопатія. Клінічний Випадок

Tseluiko¹,

Steblianko²

2022

ЕМ

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Некомпактна кардіоміопатія, згідно з класифіка-цією МКХ-10 та рекомендаціями Асоціації кардіо-логів України 2016 року, належить до розділу некла-сифікованих кардіоміопатій (І42.8) [1] і є генетично гетерогенним захворюванням. Відомі автосомно-домінантні та Х-зчеплені варіанти спадкування, що значаться як ізольовані форми некомпактного лівого шлуночка. Ізольований некомпактний лівий шлуночок (ІНЛШ) являє собою рідкісний варіант вроджених кардіоміопатій. Відомі такі форми: не-компактний міокард лівого шлуночка, сімейний, ізольований, автосомно-домінантний першого типу (ген, картований на хромосомі 18q12.1-q12.2), захворювання обумовлено мутацією альфа-дистро-бревіну, некомпактний міокард лівого шлуночка, сімейний, ізольований, автосомно-домінантний другого типу (ген, картований на хромосомі 11р15) та некомпактний міокард лівого шлуночка, сімей-ний, ізольований, Х-зчеплений (ген, картований на хромосомі Хq28), захворювання обумовлено му-тацією TAZ gene (тафазин), впливає на метаболізм кардіоліпіну [2].Захворювання виникає під час ембріональ-ного розвитку трабекулярних утворень міокарда. Пов'язане з порушенням упорядкування та ущіль-нення хаотично розташованих трабекул [3]. Підви-щена трабекулярність звичайно не зустрічається у постнатальному періоді [4]. Найчастіше ІНЛШ спостерігається в поєднанні з різними вадами сер-ця [5]. Уперше кардіоміопатія як самостійна нозо-логічна форма описана Thomas K. Chin та співавт. у 1990 році [6].Частоту ІНЛШ остаточно не встановлено. За даними Ervin N. Oechslin (2000) [7], на підставі ви-вчення ехокардіоскопій частота зустрічальності ста-новить 14 випадків на 100 тис. населення. Сімейні форми захворювання у дитячій популяції спостері-гаються у 40-50 % випадків та у 18 % серед дорос-лих. Серед чоловіків захворювання зустрічається значно частіше [7].Раніше некомпактний міокард позначали як персистуючі міокардіальні синусоїди, або як губ-частий міокард. Переважно виявляється в лівому шлуночку та може зустрічатись у правому та в обох камерах серця.Некомпактний міокард не має специфічної гіс-тологічної картини. Більшість патоморфологів від-значають підвищений фіброз трабекулярних струк-тур (рис. 1, 2) [8].Для перебігу захворювання характерний про-лонгований безсимптомний період і фіналізація тяжкою серцевою недостатністю, що потребує трансплантації серця [9,10]. Клінічні прояви за-хворювання неспецифічні, у 60-82 % випадків за-хворювання маніфестує з симптомокомплексу сер-цевої недостатності (задишка, набряки, тахікардія). Клінічна картина подібна до дилатаційної кардіо-міопатії. Смертність протягом 3-6 років становить , 58, Kharkiv, 61176, Ukraine; e-mail: Kyznetsov_I_V@mail.ru УДК 616.127-036 DOI: 10.22141/2224-0586.1.80.2017 Целуйко

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Angiographic Demonstration of Intramyocardial Sinusoids in Pulmonary-Valve Atresia with Intact Ventricular Septum and Hypoplastic Right Ventricle

Cited by 128 publications

References 1 publication

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

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

Isolated noncompaction of the myocardium

Некомпактна Кардіоміопатія. Клінічний Випадок

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