A 26-year-old woman with congenital deafness and a lifelong "seizure disorder" presented to the emergency department with 15 episodes of syncope. She was 1 week postpartum. The ECG showed normal sinus rhythm, markedly prolonged QT interval (900 ms), and remarkably large and broad T waves (Figure). In the emergency room, the patient had another syncopal episode associated with tonic-clonic activity, and monitoring showed polymorphic ventricular tachycardia (torsade de pointes), which terminated spontaneously. The patient was started on a -blocker and underwent implantation of a cardioverter-defibrillator.The diagnosis of Jervell-Lange-Nielsen syndrome, the autosomal recessive form of the long-QT syndrome associated with congenital deafness, was confirmed by identification of 2 different mutations in the potassium channel gene KCNQ1 (KvLQT1), resulting in A341V and K362R; one was a de novo mutation, and the other was inherited from her father. The peripartum period is one of increased arrhythmia risk in this syndrome. Marked QT prolongation is characteristic, but the remarkably large-amplitude T waves shown here are unusual.Marked QT-interval prolongation and remarkably large-amplitude TU complexes in a patient with Jervell-Lange-Nielsen syndrome.
SUMMARYA 65-year-old woman presented to A&E department, with acute onset central chest pain and dyspnoea. ECG showed dynamic T wave changes while 12 h troponin was elevated. A diagnosis of acute coronary syndrome was made and she underwent an inpatient coronary angiogram. Although her coronary arteries were normal, symptoms persisted and D-dimers were found to be elevated. This led to a CT pulmonary angiogram, which ruled out pulmonary embolism, but uncovered a large ascending aortic aneurysm with a contained leak. She was immediately transferred to regional cardiothoracic unit for urgent surgical intervention. This case report illustrates the importance of a good clinical history, physical examination and timely investigations. It also emphasises that not all chest pain events with elevated troponin level are due to acute coronary syndrome and that alternative diagnoses should still be considered.
BACKGROUND
Four cases of ICD system malfunction occurring within the Medtronic Marquis family over a limited time frame are reported. These cases shared a common element of defective connections between the header post and the right ventricular pace/sense terminal pin that required intervention and reconnection. Inappropriate shocks occurred in two cases. The circumstances of these cases provide important information about the potential difficulties with these devices and highlight the deficiencies of the current system in tracking and analyzing device-related problems.
Pulmonary artery aneurysm is a rare entity involving aneurysmal dilatation of the main pulmonary artery. It is associated with structural cardiac anomalies, structural vascular abnormalities, pulmonary artery hypertension, vasculitis and infection. In some cases, it can also be idiopathic. Clinical presentation may vary in different patients and are nonspecific. Treatment is usually surgical intervention once diagnosed. We have presented one such case of a patient whose Computed Tomography (CT) scan showed a large aneurysm of descending branch of pulmonary artery.
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