ABSTRACT
Objective To improve the understanding of Danon disease and the efficacy of heart transplantation by multi-disciplinary team (MDT) pattern. Methods Prior to heart transplantation, MDT consultation was performed on one case of Danon disease, an extremely rare X-chromosome dominant genetic disease. The content of consultation included surgical indication, preoperative preparation and postoperative precaution, as well as the role of MDT in the treatment of Danon disease was summarized. Results Preoperative echocardiography showed that the patient presented withdilated-phase of hypertrophic cardiomyopathy complicated with heart failure, which was considered as Danon disease with end-stage heart failure. After MDT consultation, the patient received the preoperative treatment including anti-heart failure, reduction of pulmonary artery pressure, intra-aortic balloon pump (IABP) assistance, liver protection, strengthening nutritional support, etc. The patient underwent orthotopic heart transplantation after a suitable donor was matched. The patient developed muscle weakness in the limbs 5 days after operation, which was gradually mitigated after reducing the dose of glucocorticoid. At postoperative 48 days, the patient was discharged in good condition and continually treated with triple immunosuppressive regimen after discharge. Reexamination at postoperative 6 months revealed that the patient was in good health without any abnormality in electrocardiogram and echocardiography images. Conclusions Danon disease is extremely rare and MDT pattern may enhance the efficacy and safety of treatment by heart transplantation.
ABSTRACT
Resumen: El situs inversus totalis es la inversión congénita completa de órganos torácicos y abdominales. Se presenta el caso de una paciente de 3 años sin antecedentes médicos previos, a quien en atención primaria, y por un cuadro respiratorio agudo, se evidencia el hallazgo de dextrocardia y burbuja gástrica a derecha en la radiografía de tórax, sospechándose situs inversus totalis. Fue derivada a cardiología infantil donde se confirmó el diagnóstico con un ecocardiograma transtorácico, asociado a un hallazgo de preexcitación ventricular en el electrocardiograma. Una vez resuelto el cuadro respiratorio agudo, la paciente se mantiene controlada de manera periódica en atención primaria y por especialista de manera semestral.
Abstract: Situs inversus totalis is the complete congenital inversion of thoracic and abdominal organs. We present the case of a 3-year-old girl with no previous medical history. When seen with an acute respiratory syndrome, dextrocardia and gastric bubble on the right side led to the diagnosis of Situs inversus. She was referred to infant cardiology where the diagnosis was confirmed with a transthoracic echocardiogram. In addition, the electrocardiogram identified the presence of ventricular preexitation. Once the acute respiratory symptoms subsided, the patient remains controlled periodically in primary care and by a specialist every six months. No episodes of tachycardia have been detected.
Subject(s)
Humans , Female , Child, Preschool , Situs Inversus/complications , Situs Inversus/diagnosis , Pre-Excitation Syndromes/complications , Pre-Excitation Syndromes/diagnosis , Radiography, Thoracic , Dextrocardia/complications , Dextrocardia/diagnosis , ElectrocardiographyABSTRACT
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.
Subject(s)
Humans , Bundle-Branch Block , Catheter Ablation , Pre-Excitation Syndromes , Tachycardia , Tachycardia, Supraventricular , Tachycardia, VentricularABSTRACT
Objective To investigate the association between syncope and transient suppression of sinoatrial node in patients with preexcitation syndrome and paroxysmal supraventricular tachycardia (PSVT).Methods Twelve patients (male 8,female 4;age range:19-49 years) were enrolled due to WPW(Wolff-Parkinson-White) syndrome and syncope when the tachycardia terminated.The function of sinoatrial node was evaluated with clinical electrophysiologic study,and then radiofrequency catheter ablation (RFCA) or antitachycardia pacemaker were optimized for the patients with a follow-up of 2 to 5 years.Results Electrocardiographic features of the 12 patients were: ventricular preexcitation (type A in 7 and type B in 5) and long pause when tachycardias ended up.Electrophysiologic study revealed the function of sinoatrial node was normal.Four patients received antitachycardia pacing therapy,5 underwent RFCA,and 3 received both.The 2 to 5 years follow-up found no relapse of PSVT associated syncope.Conclusion Transient suppression of sinoatrial node might explain the mechanism of syncope with preexcitation and PSVT.However,the reason why such a small group of patients differ from the majority of preexcited cases in clinical course remains to be unsettled.The first choice of prevention and treatment for these patients should be eliminating PSVT.Conventional pacemaker implantation for preventing syncope seems to be unnecessary.
ABSTRACT
We report a case in which WPW (Wolff-Parkinson-White)-type preexcitation syndrome arose unexpectedly immediately after induction of general anesthesia on a 25-yr-old man who had another rare cardiac arrhythmia, parasystole. His preoperative ECG showed ventricular bigeminy and a delta wave was observed after induction of anesthesia with fentanyl, midazolam and propofol. Anesthesia was maintained with propofol, fentanyl and nitrous oxide. The intraoperative ECG showed varying and temporary responsiveness to drugs such as atropine, lidocaine and ephedrine. After we started to infuse the dobutamine, the delta wave, ventricular bigeminy disappeared on the intraoperative ECG. We should consider the influence of anesthesia-related agents on arrhythmia, and aim to prevent and manage tachyarrhythmias caused by this syndrome.