Noncompaction of the left ventricle: a new cardiomyopathy is presented to the clinician

Moreira, Fábio Cañellas; Miglioransa, Marcelo Haertel; Mautone, Marcela; Müller, Karen Reetz; Lucchese, Fernando

doi:10.1590/s1516-31802006000100007

Cited by 17 publications

(8 citation statements)

References 18 publications

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“…Taking all the results together it was obvious that we were dealing with a case of cardiomyopathy with noncompaction of the left ventricle [1], secondary hypothyroidism and primary adrenal insufficiency. Thyroxine was started and hydrocortisone therapy was continued.…”

Section: Clinical Presentationmentioning

confidence: 99%

A case of reversible cardiomyopathy & left ventricular noncompaction

Amira¹,

Abdelbasit²,

Seidahmed³

et al. 2016

J Integr Cardiol

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This is a report of a female newborn baby who presented at the age of one hour after birth with signs of respiratory distress with marked cyanosis. The critical condition of the baby and the stormy course of the disorder highlighted the importance of starting immediate management and establishing an etiological diagnosis, as this will make the difference in the outcome and the prognosis. Clinical presentationThis was a female infant born at term after an uneventful pregnancy. Antenatal ultrasound did not show fetal anomaly and antenatal serology for infectious diseases was not significant. High vaginal swab from the mother was negative for group B streptococcus. Delivery was by elective Caesarean section because of previous section. Apgar Score was 9 and 10 at 1 and 5 minutes respectively. Birth weight was 2230 grams but the baby was vigorous with an oxygen saturation of 95% in room air at 15 minutes and was eventually sent to the normal newborn nursery.At the age of one hour the pediatric doctor was called because the baby was showing signs of respiratory distress with marked cyanosis and an oxygen saturation ranging from 70% to 80% in room air. Clinical examination revealed no signs of dysmorphism but the baby was showing respiratory distress with marked cyanosis. There were no abnormal findings on examination of the cardiovascular system. On admission to the neonatal intensive care unit (NICU) the baby was started on nasal continuous positive airway pressure (nCPAP) of 6 cm H 2 O, FiO 2 of 0.3 but the oxygen saturation (SaO 2 ) remained unchanged at 80% and below. Blood gases at that time showed a pH of 7.12, pCO 2 of 45 mm Hg and a bicarbonate of 14 mmol/I with a base deficit of -13. With the increasing requirements of oxygen the baby was shifted from nCPAP to mechanical ventilation. In spite of reaching an inspiratory pressure of 26 cm H 2 O the baby remained hypoxemic at which point a diagnosis of persistent pulmonary hypertension (PPHN) was entertained and high frequency oscillatory ventilation (HFOV) was started together with inhaled nitric oxide. Soon after admission the baby was noticed to have systemic hypotension requiring inotropic therapy. Dopamine and dobutamine were titrated to a maximum of 20 micrograms per kilogram per minute after which epinephrine and hydrocortisone were instituted consecutively because of persistent hypotension. At this point the differential diagnosis rested between cyanotic congenital heart disease, septic shock or metabolic disorder and antibiotics were started.On interviewing the family it was revealed that the parents were first-degree cousins with five living children but one of the siblings who was a male infant expired at the age of two days and the death was attributed to possible hypoxic ischemic encephalopathy, septic shock or a metabolic disorder most likely adrenal insufficiency.Taking into account the clinical presentation and the family history the baby was investigated accordingly. A chest radiograph revealed normal lung fields with no cardiomegaly. I...

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Section: Clinical Presentationmentioning

confidence: 99%

A case of reversible cardiomyopathy & left ventricular noncompaction

Amira¹,

Abdelbasit²,

Seidahmed³

et al. 2016

J Integr Cardiol

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“…In our review, 1 we had not identifi ed any acquired left ventricular hypertrabeculation/noncompaction (LVHT) cases, and Dr. Finsterer's contribution regarding the instances in which such cases can occur is of great value. 2 In fact, it is important to investigate neuromuscular disorders in patients for whom a diagnosis of apparent LVHT alone is proposed.…”

Section: Responsementioning

confidence: 99%

Left ventricular hypertrabeculation/noncompaction: a cardiac manifestation of myopathy?

Finsterer

Stöllberger

2007

Sao Paulo Med. J.

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“…For example in the case of hypertrophic cardiomyopathy, it is known that children are at higher risk for arrhythmias and sudden death than adults. In most of the cases an ECG tracing is required and this is sufficient for an accurate diagnosis, whereas in some cases, a more sophisticated modality is required [2], [3].…”

Section: Introductionmentioning

confidence: 99%

“…The holter monitor has proved insufficient because it is limited to 24 or 48 hours of recording during which the patient may be asymptomatic. We care for a group of such children, some of whom are at imminent risk of sudden death [2], [3].…”

Section: Introductionmentioning

confidence: 99%

An m-Health Monitoring System for Children with Suspected Arrhythmias

Kyriacou

Pattichis

et al. 2007

2007 29th Annual International Conference of the IEEE Engineering in Medicine and Biology Society

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Advances in wireless communications and networking technologies as well as computer and medical technologies, enable the development of small size, power efficient and more reliable medical multi-parameter recording systems, which can be used for continuous monitoring of patients. Through this paper we present the basic architecture and initial development steps of an m-Health monitoring system that will be used in order to monitor children with suspected cardiac arrhythmias. The proposed system will be based on sensor networks, in order to monitor a subject while being in a predefined area like his/her house; while a module based on PDAs and wearable ECG recorders will be used in order to extent the coverage outside the patient's house. The system will be based on a variable sampling rate to conserve power for the possible arrhythmia episode. The system design has been completed, the hardware specifications have been decided and currently the system is going through the development phase.

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Noncompaction of the left ventricle: a new cardiomyopathy is presented to the clinician

Cited by 17 publications

References 18 publications

A case of reversible cardiomyopathy & left ventricular noncompaction

A case of reversible cardiomyopathy & left ventricular noncompaction

Left ventricular hypertrabeculation/noncompaction: a cardiac manifestation of myopathy?

An m-Health Monitoring System for Children with Suspected Arrhythmias

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