Humans ; Wolff-Parkinson-White Syndrome/diagnosis* ; Electrocardiography ; Athletes

Objective: To review the clinical data of 7 patients with Danon disease and analyze their clinical characteristics. Methods: The medical records of 7 patients with Danon disease, who were hospitalized in Peking Union Medical College Hospital of Chinese Academy of Medical Sciences from April 2008 to July 2021, were reviewed and summarized, of which 6 cases were diagnosed as Danon disease by lysosomal-associated membrane protein-2 (LAMP-2) gene mutation detection and 1 case was diagnosed by clinicopathological features. Clinical manifestations, biochemical indexes, electrocardiogram, echocardiography, skeletal muscle and myocardial biopsy and gene detection results were analyzed, and patients received clinical follow-up after discharge. Results: Six patients were male and average age was (15.4±3.5) years and the average follow-up time was (27.7±17.0) months. The main clinical manifestations were myocardial hypertrophy (6/7), decreased myodynamia (2/7) and poor academic performance (3/7). Electrocardiogram features included pre-excitation syndrome (6/7) and left ventricular hypertrophy (7/7). Echocardiography examination evidenced myocardial hypertrophy (6/7), and left ventricular dilatation and systolic dysfunction during the disease course (1/7). The results of skeletal muscle biopsy in 6 patients were consistent with autophagy vacuolar myopathy. Subendocardial myocardial biopsy was performed in 3 patients, and a large amount of glycogen deposition with autophagosome formation was found in cardiomyocytes. LAMP-2 gene was detected in 6 patients, and missense mutations were found in all these patients. During the follow-up period, implantable cardioverter defibrillator implantation was performed in 1 patient because of high atrioventricular block 4 years after diagnosis, and there was no death or hospitalization for cardiovascular events in the other patients. Conclusion: The main clinical manifestations of Danon disease are cardiomyopathy, myopathy and mental retardation. Pre-excitation syndrome is a common electrocardiographic manifestation. Autophagy vacuoles can be seen in skeletal muscle and myocardial pathological biopsies. LAMP-2 gene mutation analysis is helpful in the diagnose of this disease.
Adolescent ; Child ; Female ; Humans ; Male ; Cardiomyopathies/etiology* ; Glycogen Storage Disease Type IIb/complications* ; Hypertrophy, Left Ventricular/etiology* ; Lysosomal-Associated Membrane Protein 2/genetics* ; Pre-Excitation Syndromes/genetics*

No abstract available.
Acidosis, Lactic ; Brain Diseases ; Humans ; Myoclonic Epilepsy, Juvenile ; Wolff-Parkinson-White Syndrome

Congenital cardiovascular anomalies, such as dextrocardia, persistent left superior vena cava (SVC), and pulmonary artery (PA) sling, are rare disorders. These congenital anomalies can occur alone, or coincide with other congenital malformations. In the majority of cases, congenital anomalies are detected early in life by certain signs and symptoms. A 56-year-old man with no previous medical history was admitted due to recurrent wide QRS complex tachycardia with hemodynamic collapse. A chest radiograph showed dextrocardia. After synchronized cardioversion, an electrocardiogram revealed Wolff-Parkinson-White (WPW) syndrome. Persistent left SVC, PA sling, and right tracheal bronchus were also detected by a chest computed tomography (CT) scan. He was diagnosed with paroxysmal supraventricular tachycardia (PSVT) associated with WPW syndrome, and underwent radiofrequency ablation. We reported the first case of situs solitus dextrocardia coexisting with persistent left SVC, PA sling and right tracheal bronchus presented with WPW and PSVT in a middle-aged adult. In patients with a cardiovascular anomaly, clinicians should consider thorough evaluation of possibly combined cardiovascular and airway malformations and cardiac dysrhythmia.
Adult* ; Arrhythmias, Cardiac ; Bronchi ; Catheter Ablation ; Dextrocardia ; Electric Countershock ; Electrocardiography ; Hemodynamics ; Humans ; Middle Aged ; Pulmonary Artery ; Radiography, Thoracic ; Tachycardia ; Tachycardia, Supraventricular ; Thorax ; Vena Cava, Superior ; Wolff-Parkinson-White Syndrome*

Double tachycardia is defined as the coexistence of supraventricular tachycardia (SVT) and ventricular tachycardia (VT). In clinical practice, incidentally encountered double tachycardia is difficult to diagnose during electrophysiology study without prior documentation of both tachycardias. SVT can be confused with VT because SVT sometimes show an aberrant conduction depending on heart rate. In the present case, a patient with Wolff-Parkinson-White syndrome exhibited atrioventricular reentrant tachycardia (AVRT) via a concealed bypass tract and idiopathic left ventricular tachycardia (ILVT), which were difficult to discriminate because of their similar cycle length and interrelationship.
Electrophysiology ; Heart Rate ; Humans ; Tachycardia* ; Tachycardia, Supraventricular ; Tachycardia, Ventricular* ; Wolff-Parkinson-White Syndrome

Wide QRS complex tachycardia with a left bundle branch block pattern can be caused by supraventricular tachycardia with aberrant conduction, preexcitation syndrome mediated through a right-sided accessory pathway, and/or ventricular tachycardia. The use of atrial pacing maneuvers can be beneficial for unmasking minimal preexcitation to differentiate between these conditions. Here, we report a case of successful radiofrequency catheter ablation of a Mahaim fiber in a patient with wide QRS complex tachycardia.
Bundle-Branch Block ; Catheter Ablation ; Humans ; Pre-Excitation Syndromes* ; Tachycardia ; Tachycardia, Supraventricular ; Tachycardia, Ventricular

In patients with Ebstein's anomaly, the localization of accessory pathways may be impeded by abnormal local electrograms recorded along the atrialized right ventricle and by the presence of multiple accessory pathways. We report a case of 50-year-old man diagnosed with Ebstein's anomaly with Wolff-Parkinson-White syndrome who presented with recurrent palpitations. He was referred to our institution for radiofrequency catheter ablation of paroxysmal supraventricular tachycardia. Transthoracic echocardiography revealed the tricuspid valve displaced into the right ventricle, consistent with Ebstein's anomaly. The electrophysiology study showed a right posterolateral accessory pathway. The optimal ablation site was located not in the atrioventricular line of the atrialized ventricular portion, but in the original atrioventricular line.
Accessory Atrioventricular Bundle ; Catheter Ablation ; Ebstein Anomaly* ; Echocardiography ; Electrophysiology ; Heart Ventricles ; Humans ; Middle Aged ; Tachycardia, Supraventricular ; Tricuspid Valve ; Wolff-Parkinson-White Syndrome

Among patients with Wolff-Parkinson-White syndrome, atrioventricular reciprocating tachycardia (AVRT) and atrioventricular nodal reentrant tachycardia (AVNRT) can coexist in a single patient. Direct transition of both tachycardias is rare; however, it can occur after premature atrial or ventricular activity if the cycle lengths of the two tachycardias are similar. Furthermore, persistent atrial activation by an accessory pathway (AP) located outside of the AV node during ongoing AVNRT is also rare. This article describes a case of uncommon atrial activation by an AP during AVNRT and gradual transition of the two supraventricular tachycardias without any preceding atrial or ventricular activity in a patient with preexcitation syndrome.
Atrioventricular Node ; Humans ; Pre-Excitation Syndromes ; Tachycardia* ; Tachycardia, Atrioventricular Nodal Reentry ; Tachycardia, Paroxysmal ; Tachycardia, Reciprocating ; Tachycardia, Supraventricular ; Wolff-Parkinson-White Syndrome*

Wolff Parkinson White (WPW) syndrome is a condition in which there is an aberrant conduction pathway between the atria and ventricles, resulting in tachycardia. A 42-year-old patient, who was treated for WPW syndrome previously, presented with chronic somatic pain. With her cardiac condition in mind, she was thoroughly worked up for a recurrence of disease. As part of routine screening of all patients at our pain clinic, she was found to have severe depression as per the Patient Health Questionnaire–9 (PHQ–9) criteria. After ruling out sinister causes, she was treated for depression using oral Duloxetine and counselling. This led to resolution of symptoms, and improved her mood and functional capability. This case highlights the use of psychological screening tools and diligent examination in scenarios as confusing as the one presented here. Addressing the psychological aspects of pain and adopting a holistic approach are as important as treatment of the primary pathology.
Adult ; Chest Pain* ; Chronic Pain ; Depression* ; Duloxetine Hydrochloride ; Humans ; Mass Screening ; Nociceptive Pain ; Pain Clinics ; Pathology ; Recurrence ; Tachycardia ; Thorax* ; Wolff-Parkinson-White Syndrome*

Wolff Parkinson White (WPW) syndrome is a condition in which there is an aberrant conduction pathway between the atria and ventricles, resulting in tachycardia. A 42-year-old patient, who was treated for WPW syndrome previously, presented with chronic somatic pain. With her cardiac condition in mind, she was thoroughly worked up for a recurrence of disease. As part of routine screening of all patients at our pain clinic, she was found to have severe depression as per the Patient Health Questionnaire–9 (PHQ–9) criteria. After ruling out sinister causes, she was treated for depression using oral Duloxetine and counselling. This led to resolution of symptoms, and improved her mood and functional capability. This case highlights the use of psychological screening tools and diligent examination in scenarios as confusing as the one presented here. Addressing the psychological aspects of pain and adopting a holistic approach are as important as treatment of the primary pathology.
Adult ; Chest Pain* ; Chronic Pain ; Depression* ; Duloxetine Hydrochloride ; Humans ; Mass Screening ; Nociceptive Pain ; Pain Clinics ; Pathology ; Recurrence ; Tachycardia ; Thorax* ; Wolff-Parkinson-White Syndrome*