Chest
Selected ReportsCardiomyopathic Lentiginosis/LEOPARD Syndrome Presenting as Sudden Cardiac Arrest
Section snippets
CASE REPORT
An apparently healthy 26-year-old man was admitted to the ICU after successful resuscitation from documented ventricular fibrillation sustained during dancing the polka. A comprehensive past medical history could be obtained from his mother: “moles” had erupted during childhood; a dermatologist had diagnosed nevi and had recommended surveillance. In 1980, an operation was performed for an undescended testicle; an aneurysm of the left femoral vein was excised in 1988. In 1991, ultrasound studies
COMMENT
Sudden ventricular fibrillation is uncommon in young adults. Unlike in the elderly, coronary heart disease is rarely the cause. Instead, myocarditis, cardiomyopathy, right ventricular dysplasia, use of illicit drugs, and anomalies of the QT interval prevail. Hypertrophic cardiomyopathy is a key feature of cardiomyopathic lentiginosis.6 Interestingly, sudden death has been mentioned in previous reports of the disorder.7
In this case, cardiomyopathy was revealed as the most likely cause and
CONCLUSION
Lentiginosis must prompt thorough evaluation since it may be part of a multifaceted syndrome that cannot only be associated with considerable morbidity but may even place patients at risk for sudden death. Although identification of patients in need of prophylactic treatment will remain difficult, pacer-cardioverter-defibrillator device therapy is believed to be indicated and beneficial in survivors of out-of-hospital ventricular fibrillation.
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SHP2 sails from physiology to pathology
2015, European Journal of Medical GeneticsCitation Excerpt :NS-ML-associated HCM in general affects the left ventricle, with outflow obstruction for 40% of cases, and can be congenital or develop during childhood. Cardiological follow-up is required given the risk of heart failure or sudden death (Lauriol et al., 2014; Limongelli et al., 2007, 2008; Woywodt et al., 1998). Lentigines are a major feature of NS-ML, found in more than 90% of cases (Martinez-Quintana and Rodriguez-Gonzalez, 2012).
Anesthesia and LEOPARD syndrome: A review of forty-nine anesthetic exposures
2014, Journal of Cardiothoracic and Vascular AnesthesiaCitation Excerpt :His heart failure and pulmonary hypertension were managed medically and, during neurosurgical procedures, required vasopressor and prostaglandin infusion to maintain cardiac output. Electrocardiographic abnormalities are frequent in LS patients (Table 2), and while some are benign and asymptomatic, these patients may have the propensity for life-threatening arrhythmias including ventricular fibrillation and sudden death.17,18 Indeed, most of the complications in the authors’ patients were arrhythmias.
LEOPARD syndrome: A variant of noonan syndrome strongly associated with hypertrophic cardiomyopathy
2013, Revista Espanola de CardiologiaNeonatal atrial tachycardia: Suggestive clinical sign of Costello syndrome
2011, Archives de PediatriePTPN11 mutations in LEOPARD syndrome: Report of four cases in Taiwan
2009, Journal of the Formosan Medical AssociationPrevalence and Clinical Significance of Cardiovascular Abnormalities in Patients With the LEOPARD Syndrome
2007, American Journal of CardiologyCitation Excerpt :Nevertheless, we observed 4 fatal events in our study population. Sporadic cases reported sudden death in patients with LS associated with LV hypertrophy.3,19 In contrast, several studies described the clinical outcomes and investigated risk assessment in patients with familial hypertrophic cardiomyopathy.20