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Article: Anesthetic experience of patient with isolated left ventricular noncompaction: a case report.

Kim, Doyeon / Kim, Eunhee / Lee, Jong-Hwan / Kim, Chung Su / Lee, Sangmin Maria / Lee, Jung Eun

2016 Volume 69, Issue 3, Page(s) 275–278

Abstract: Isolated left ventricular noncompaction (LVNC) is a rare primary genetic cardiomyopathy ... characterized by prominent trabeculation of the left ventricular wall and intertrabecular recesses ... of heart failure, systemic thromboembolic events, and fatal left ventricular arrhythmias. We conducted real time ...

Abstract	Isolated left ventricular noncompaction (LVNC) is a rare primary genetic cardiomyopathy characterized by prominent trabeculation of the left ventricular wall and intertrabecular recesses. Perioperative management of the patient with LVNC might be challenging due to the clinical symptoms of heart failure, systemic thromboembolic events, and fatal left ventricular arrhythmias. We conducted real time intraoperative transesophageal echocardiography in a patient with LVNC undergoing general anesthesia for ovarian cystectomy.
Language	English
Publishing date	2016-06
Publishing country	Korea (South)
Document type	Journal Article
ZDB-ID	2557340-8
ISSN	2005-7563 ; 2005-6419
ISSN (online)	2005-7563
ISSN	2005-6419
DOI	10.4097/kjae.2016.69.3.275
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Article ; Online: Isolated ventricular noncompaction: a case report.

Bento, João / Monteiro, Filipe / Sargento, Luis / Vizcaino, Joaquin / Monteiro, Jorge / Azevedo, Pilar / Brum, Gabriela

Cases journal

2009 Volume 2, Page(s) 9312

Abstract: Isolated ventricular noncompaction is an extremely rare cardiomyopathy, not fully clarified.It is ... asymptomatic young male, which was the initial presentation of an isolated ventricular noncompaction.A brief ... consist on a few case studies.Doppler ecocardiogram is considered the reference method for diagnosis ...

Abstract	Isolated ventricular noncompaction is an extremely rare cardiomyopathy, not fully clarified.It is characterized by persistent embryonic myocardium morphology without associated cardiac abnormalities.Since first description in 1984, few clinical studies were done. Data in the literature are lacking and most reports consist on a few case studies.Doppler ecocardiogram is considered the reference method for diagnosis.Diagnosis remains difficult since there are similarities with other cardiac defects, clinical manifestations are non-specific and echocardiographic criteria are not universally accepted.As a consequence diagnosis may be easily missed.Moreover, clinical and echocardiographic features were just recently clarified.Treatment is directed towards important clinical manifestations (heart failure, arrhythmias and embolic events).We present a clinical case of severe cardio-respiratory failure in previously healthy and asymptomatic young male, which was the initial presentation of an isolated ventricular noncompaction.A brief review of available literature is done concerning to this case study.
Language	English
Publishing date	2009-12-11
Publishing country	England
Document type	Journal Article
ZDB-ID	2431132-7
ISSN	1757-1626 ; 1757-1626
ISSN (online)	1757-1626
ISSN	1757-1626
DOI	10.1186/1757-1626-2-9312
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Article ; Online: Acute Kidney Infarction Due to Left Ventricular Thrombus Embolization In Patient with Isolated Left Ventricular Noncompaction: A Case Report.

Xu, Liqian / Yang, Ji / Yang, Yunmei

The heart surgery forum

2017 Volume 20, Issue 6, Page(s) E252–E255

Abstract: Noncompaction of the ventricular myocardium (NVM) is a rare congenital cardiomyopathy that is ... left ventricular noncompaction (LVNC), because lesions are mainly in the left ventricle. The main clinical ... of normal embryogenesis of the endocardium and myocardium. It is also referred to as isolated ...

Abstract	Noncompaction of the ventricular myocardium (NVM) is a rare congenital cardiomyopathy that is characterized by multiple prominent trabeculations and deep intertrabecular recesses, and occurs due to arrest of normal embryogenesis of the endocardium and myocardium. It is also referred to as isolated left ventricular noncompaction (LVNC), because lesions are mainly in the left ventricle. The main clinical manifestations are heart failure, arrhythmia, systemic embolism, and sudden death. Systemic embolism is related to the occurrence of atrial arrhythmias or thrombus formation in the left ventricle. Most resulting thromboembolisms are cerebral or in the arteries of the lower limbs, and renal artery embolisms are rare. There are reports of a few previous cases of renal infarction with diagnoses of NVM on echocardiography, but a thrombus from the left ventricle has never been identified as the cause of a renal artery embolism. This paper reports a 53-year-old male who was admitted to our hospital for LVNC and renal infarction. He had a history of atrial fibrillation 3 years previously. We diagnosed LVNC with a thrombus in the left ventricle using echocardiography. The patient was discharged after renal replacement therapy and treatment with an anticoagulant. During the 2 years of follow-up, his condition remained stable.
MeSH term(s)	Anticoagulants/therapeutic use ; Diagnosis, Differential ; Echocardiography ; Embolism/complications ; Embolism/diagnosis ; Embolism/therapy ; Follow-Up Studies ; Heart Defects, Congenital/complications ; Heart Defects, Congenital/diagnosis ; Heart Diseases/complications ; Heart Diseases/diagnosis ; Heart Diseases/therapy ; Heart Ventricles ; Humans ; Infarction/diagnosis ; Infarction/etiology ; Infarction/therapy ; Kidney/blood supply ; Male ; Middle Aged ; Renal Artery ; Renal Replacement Therapy ; Thrombolytic Therapy ; Thrombosis
Chemical Substances	Anticoagulants
Language	English
Publishing date	2017--07
Publishing country	United States
Document type	Case Reports ; Journal Article
ZDB-ID	2069188-9
ISSN	1522-6662 ; 1098-3511
ISSN (online)	1522-6662
ISSN	1098-3511
DOI	10.1532/hsf.1608
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Article ; Online: A case report of isolated right ventricular noncompaction with mutation of ACVRL1: a new cause of noncompaction of ventricular myocardium?

Yu, Bo / Shi, Kun / Wen, Yang / Yang, Yanfeng

BMC cardiovascular disorders

2023 Volume 23, Issue 1, Page(s) 224

Abstract: ... associated with genetic mutations and nongenetic factors, among which the isolated right ventricular ... right ventricular failure caused by ACVRL1 mutation, or they happened in the same case coincidently. ... hereditary hemorrhagic telangiectasia (HHT2), and there's no NVM reported to be associated with ACVRL1 mutation.: Case presentation ...

Abstract	Background: Noncompaction of ventricular myocardium(NVM) is a rare kind of cardiomyopathy associated with genetic mutations and nongenetic factors, among which the isolated right ventricular noncompaction (iRVNC) is the most rare type. ACVRL1 is the pathogenic gene of type 2 hereditary hemorrhagic telangiectasia (HHT2), and there's no NVM reported to be associated with ACVRL1 mutation. Case presentation: This is a rare case diagnosed as iRVNC and pulmonary hypertention with ACVRL1 mutation detected. Conclusion: iRVNC in this case may be due to ACVRL1 mutation, secondary to pulmonary hypertention and right ventricular failure caused by ACVRL1 mutation, or they happened in the same case coincidently.
MeSH term(s)	Humans ; Telangiectasia, Hereditary Hemorrhagic/genetics ; Telangiectasia, Hereditary Hemorrhagic/pathology ; Mutation ; Lung ; Heart Failure ; Myocardium/pathology ; Activin Receptors, Type II/genetics
Chemical Substances	ACVRL1 protein, human (EC 2.7.11.30) ; Activin Receptors, Type II (EC 2.7.11.30)
Language	English
Publishing date	2023-04-29
Publishing country	England
Document type	Case Reports ; Journal Article
ZDB-ID	2059859-2
ISSN	1471-2261 ; 1471-2261
ISSN (online)	1471-2261
ISSN	1471-2261
DOI	10.1186/s12872-023-03132-y
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Article: Isolated Left Ventricular Noncompaction Presenting With Heart Failure With Reduced Ejection Fraction and Intrahospital Cardiac Arrest: A Case Report and Literature Review.

Gonçalves, Filipa Madalena F / Batista, Marta / Campos, Ana Luísa / Costa, Magda / Cotter, Jorge

Cureus

2022 Volume 14, Issue 10, Page(s) e30559

Abstract: Left ventricular noncompaction (LVNC) is characterized by a bilayered appearance of the myocardium ... and systemic embolisms are the most frequent cardiovascular complications. We describe a case of a 53 ... conduction abnormalities, tachyarrhythmia, and sudden cardiac death. Heart failure, ventricular arrhythmias ...

Abstract	Left ventricular noncompaction (LVNC) is characterized by a bilayered appearance of the myocardium with excessive trabeculations and deep intertrabecular recesses. Manifestations of this condition are widely variable, ranging from incidental findings in asymptomatic individuals to symptomatic heart failure, conduction abnormalities, tachyarrhythmia, and sudden cardiac death. Heart failure, ventricular arrhythmias, and systemic embolisms are the most frequent cardiovascular complications. We describe a case of a 53-year-old woman who presented to the emergency department with acute presentation of previously unknown heart failure with reduced ejection fraction and was diagnosed with LVNC. During hospitalization, the patient presented a defibrillated cardiac arrest rhythm, which was resuscitated after six minutes, and then treated with the placement of an implantable cardioverter defibrillator. After two years of follow-up with optimized medical therapy, the patient currently is asymptomatic and with a preserved ejection fraction.
Language	English
Publishing date	2022-10-21
Publishing country	United States
Document type	Case Reports
ZDB-ID	2747273-5
ISSN	2168-8184
ISSN	2168-8184
DOI	10.7759/cureus.30559
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Article ; Online: Isolated left ventricular apical hypoplasia with myocardial non-compaction: a case report.

Skidan, Viktoria I / Kuznetsova, Tatiana / Pavlyukova, Elena N / Nartsissova, Galina P

European heart journal. Case reports

2019 Volume 4, Issue 1, Page(s) 1–6

Abstract: Background: Isolated left ventricular apical hypoplasia (ILVAH) is a rare congenital cardiac ... atrial fibrillation (AF), or malignant ventricular tachycardia in adults.: Case summary: A 32-years-old man ... left ventricular myocardium non-compaction was observed in this young adult who presented with symptomatic HF and ...

Abstract	Background: Isolated left ventricular apical hypoplasia (ILVAH) is a rare congenital cardiac abnormality, which might result in severe symptomatic heart failure (HF) with pulmonary hypertension, atrial fibrillation (AF), or malignant ventricular tachycardia in adults. Case summary: A 32-years-old man presented with exertional dyspnoea New York Heart Association Class II and persistent AF. Echocardiography and cardiac magnetic resonance showed the presence of (i) spherical remodelling of the left ventricle (LV) with impaired contractile function (three-dimensional ejection fraction, EF 32%); (ii) substitution of apical myocardium by fatty tissue; (iii) abnormal origin of a papillary muscle network; and (iv) an elongated right ventricle, compatible with ILVAH. In addition, non-compacted endomyocardial layer of the LV was observed. Because of a high risk of sudden cardiac death in symptomatic HF patients with reduced EF, an implantable cardioverter-defibrillator was placed which followed by pulmonary vein isolation. After the procedures and restoration of sinus rhythm, the patient demonstrated improvement in HF symptoms and exercise tolerance. This was accompanied by an enhancement of left and right ventricular systolic function by echocardiography. At 6-month, 1, and 2-year follow-up the clinical conditions of the patient and echocardiographic findings remained stable. Discussion: A rare combination of ILVAH and left ventricular myocardium non-compaction was observed in this young adult who presented with symptomatic HF and persistent AF. The use of consecutive invasive cardiac procedures leads to restoration of sinus rhythm, the improvement of myocardial contractility and clinical manifestation of HF.
Language	English
Publishing date	2019-12-31
Publishing country	England
Document type	Case Reports
ISSN	2514-2119
ISSN (online)	2514-2119
DOI	10.1093/ehjcr/ytz215
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Article ; Online: Prenatal ultrasound diagnosis of fetal isolated right ventricular noncompaction with pulmonary artery sling: A rare case report.

Zhang, Jiaqi / Wang, Yu / Feng, Wei / Wu, You

Echocardiography (Mount Kisco, N.Y.)

2019 Volume 36, Issue 11, Page(s) 2118–2121

Abstract: ... as isolated right ventricular noncompaction (IRVNC) is rare. Pulmonary artery sling (PAS) is a rare condition ... we report a case of IRVNC complicated with PAS that was diagnosed prenatally at 30 weeks gestation and ... Noncompaction of the ventricular myocardium (NVM), also known as spongy myocardium, is a rare type ...

Abstract	Noncompaction of the ventricular myocardium (NVM), also known as spongy myocardium, is a rare type of cardiomyopathy that has a serious impact on fetuses, children, and adults. NVM mainly affects the left ventricle, as isolated right ventricular noncompaction (IRVNC) is rare. Pulmonary artery sling (PAS) is a rare condition in which the left pulmonary artery anomalously originates from a normal positioned right pulmonary artery, and only a few studies have reported PAS in fetuses. Fetal IRVNC complicated with PAS has not been reported yet. Here, we report a case of IRVNC complicated with PAS that was diagnosed prenatally at 30 weeks gestation and confirmed by postpartum anatomy and pathology.
MeSH term(s)	Adult ; Echocardiography, Doppler, Color/methods ; Fatal Outcome ; Female ; Fetal Heart/diagnostic imaging ; Gestational Age ; Heart Ventricles/abnormalities ; Heart Ventricles/diagnostic imaging ; Heart Ventricles/embryology ; Humans ; Infant, Newborn ; Isolated Noncompaction of the Ventricular Myocardium/diagnosis ; Isolated Noncompaction of the Ventricular Myocardium/embryology ; Pregnancy ; Pulmonary Artery/abnormalities ; Pulmonary Artery/diagnostic imaging ; Pulmonary Artery/embryology ; Rare Diseases ; Ultrasonography, Prenatal/methods ; Vascular Malformations/diagnosis ; Vascular Malformations/embryology
Language	English
Publishing date	2019-11-06
Publishing country	United States
Document type	Case Reports ; Research Support, Non-U.S. Gov't
ZDB-ID	843645-9
ISSN	1540-8175 ; 0742-2822
ISSN (online)	1540-8175
ISSN	0742-2822
DOI	10.1111/echo.14528
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Zs.A 2170: Show issues			Location: Je nach Verfügbarkeit (siehe Angabe bei Bestand) bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular Jg. 1995 - 2021: Lesesall (1.OG) ab Jg. 2022: Lesesaal (EG)
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Article ; Online: Sudden Death in a Case of Isolated Left Ventricular Noncompaction: An Autopsy Case Report.

Lad, Shilpa K / Amonkar, Gayathri

The American journal of forensic medicine and pathology

2015 Volume 36, Issue 4, Page(s) 249–250

Abstract: We report a case of isolated left ventricular noncompaction in a 15-year-old girl, who presented ...

Abstract	We report a case of isolated left ventricular noncompaction in a 15-year-old girl, who presented with sudden death. There were no associated anomalies or history of cardiac disease.
MeSH term(s)	Adolescent ; Death, Sudden/etiology ; Female ; Forensic Pathology ; Heart Ventricles/pathology ; Humans ; Hypertrophy, Left Ventricular/pathology ; Hypertrophy, Right Ventricular/pathology ; Isolated Noncompaction of the Ventricular Myocardium/pathology
Language	English
Publishing date	2015-12
Publishing country	United States
Document type	Case Reports ; Journal Article
ZDB-ID	604537-6
ISSN	1533-404X ; 0195-7910
ISSN (online)	1533-404X
ISSN	0195-7910
DOI	10.1097/PAF.0000000000000191
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Zs.A 1613: Show issues

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Article: Different Manifestations in Familial Isolated Left Ventricular Non-compaction: Two Case Reports and Literature Review.

Al Hussein, Hamida / Al Hussein, Hussam / Stroe, Valentin / Harpa, Marius / Ghiragosian, Claudiu / Goia, Cristina Maria / Opris, Carmen Elena / Suciu, Horatiu

Frontiers in pediatrics

2020 Volume 8, Page(s) 370

Abstract: ... a case of mother and son diagnosed with isolated LVNC (ILVNC). A 4-years-old male patient, diagnosed at 3 ... Left ventricular non-compaction (LVNC) is a form of cardiomyopathy characterized by prominent ... trabeculae and deep intertrabecular recesses which form a distinct "non-compacted" layer in the myocardium ...

Abstract	Left ventricular non-compaction (LVNC) is a form of cardiomyopathy characterized by prominent trabeculae and deep intertrabecular recesses which form a distinct "non-compacted" layer in the myocardium. It results from intrauterine arrest of the compaction process of the left ventricular myocardium. Clinical manifestations vary from asymptomatic to heart failure (HF), arrhythmias, or thromboembolic events. We present a case of mother and son diagnosed with isolated LVNC (ILVNC). A 4-years-old male patient, diagnosed at 3 months with ILVNC, and NYHA functional class IV HF, was admitted to the Emergency Institute for Cardiovascular Diseases and Transplantation of Targu Mures, Romania, for cardiologic reevaluation, and diagnosis confirmation. ILVNC was confirmed using echocardiography, revealing a non-compaction to compaction (NC/C) ratio of > 2.7. His evolution was stationary until the age of 8 years, when severe pneumonia caused hemodynamic decompensation, and he was listed for heart transplantation (HT). The patient underwent HT at the age of 11 years with favorable postoperative outcome. Meanwhile, a 22-years-old female patient, mother of the aforementioned patient, was also admitted to our institute due to severe fatigue, dyspnea, and recurrent palpitations with multiple implantable cardioverter defibrillator (ICD) shock delivery. Extensive medical history revealed that a presumptive ILVNC diagnosis was established when she was 11 years old. She was asymptomatic until 18 years old, when 3 months post-partum, she developed NYHA functional class III HF, and subsequently underwent ICD implantation. Her diagnosis was confirmed using multi-detector computed tomography angiography, which revealed a NC/C ratio of > 3.3. ICD adjustments were carried out with a favorable evolution under chronic drug therapy. The last evaluation, at 27 years old, revealed that she was in NYHA functional class II HF. In conclusion, ILVNC, even when familial, can present different clinical pictures and therefore requires different medical approaches.
Language	English
Publishing date	2020-07-07
Publishing country	Switzerland
Document type	Case Reports
ZDB-ID	2711999-3
ISSN	2296-2360
ISSN	2296-2360
DOI	10.3389/fped.2020.00370
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Article: Case report: Ablation of diffuse inter-trabecular substrate in a patient with isolated ventricular non-compaction.

Jackson, Nicholas / King, Benjamin / Viswanathan, Karthik / Downar, Eugene / Spears, Danna

Indian pacing and electrophysiology journal

2015 Volume 15, Issue 3, Page(s) 162–164

Language	English
Publishing date	2015-09-16
Publishing country	Netherlands
Document type	Journal Article
ZDB-ID	2123606-9
ISSN	0972-6292
ISSN	0972-6292
DOI	10.1016/j.ipej.2015.09.002
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