Alahmadi Mohamed H., Siddiqui Waqas J.
University of Health Sciences, Lahore
Drexel University
Cardiac rhabdomyoma is a rare, benign mesenchymal tumor of striated muscle origin and represents the most common primary cardiac tumor in the pediatric population, typically diagnosed before the age of 1. While rhabdomyomas can occur in both cardiac and extracardiac locations, the extracardiac forms—classified as adult, fetal, and germ cell tumors—most often involve the head and neck. Cardiac rhabdomyomas are histologically regarded as hamartomas and strongly associated with tuberous sclerosis complex, or Bourneville disease. They most frequently arise within the ventricular myocardium, followed by the atria, cavoatrial junction, and epicardial surface in descending order of prevalence. These tumors are often multiple and tend to regress spontaneously, making conservative management appropriate unless symptoms occur. Clinical manifestations result primarily from obstruction of cardiac inflow or outflow tracts, leading to congestive heart failure, or from arrhythmogenic effects. The latter can range from bradycardia due to sinus or atrioventricular (AV) node dysfunction to more complex tachyarrhythmias, including atrial or ventricular tachycardia, AV nodal reentrant tachycardia, and ventricular preexcitation.
心脏横纹肌瘤是一种罕见的、起源于横纹肌的良性间叶组织肿瘤，是儿童最常见的原发性心脏肿瘤，通常在1岁前被诊断出来。虽然横纹肌瘤可发生于心脏内和心脏外，但心脏外的形式（分为成人型、胎儿型和生殖细胞肿瘤）最常累及头颈部。心脏横纹肌瘤在组织学上被视为错构瘤，与结节性硬化症（又称博恩维尔病）密切相关。它们最常出现在心室心肌内，其次是心房、腔静脉心房交界处和心外膜表面，按发病率从高到低排列。这些肿瘤通常是多发性的，并且往往会自发消退，因此除非出现症状，否则保守治疗是合适的。临床表现主要由心脏流入或流出道梗阻导致充血性心力衰竭引起，或由致心律失常作用引起。后者的范围从窦性或房室（AV）结功能障碍导致的心动过缓到更复杂的快速心律失常，包括房性或室性心动过速、房室结折返性心动过速和心室预激。
