This study reviewed different types of primary cardiac and mediastinal tumors in infants and children as well as their clinical presentation and management. Altogether, 34 consecutive patients followed from 1976 through December 2005 were analyzed. Of these 34 patients, 14 (41%) underwent surgery and 20 (59%) with rhabdomyomas were managed conservatively. Histologic examination of the surgically resected tumors showed teratoma in four patients, rhabdomyoma in four patients, and hemangioma in two patients. Myxoma, myofibroma, neuroblastoma, and malignant fibrous histiocytoma were encountered in one patient each. Follow-up evaluation was completed for 97% of the patients and extended up to 30 years. Half of the nonsurgical patients with rhabdomyoma showed partial or complete spontaneous regression. One patient died after resection of a malignant histiocytoma, and one patient required a tumor-related reoperation. Freedom from tumor-related reoperation after 10 years was 91% +/- 8.7%. Of the survivors, 85% were New York Heart Association (NYHA) class 1, and 100% had sinus rhythm. Spontaneous tumor regression is common in rhabdomyoma and surgery, and is indicated only for symptomatic patients with hemodynamically significant intracardiac obstruction. For all other benign primary cardiac tumors, complete resection usually can be accomplished with good results. Patients with giant tumor masses compressing or infiltrating the heart frequently cannot undergo complete resection. For these patients, restoration/preservation of sufficient heart function is the primary goal. Malignant tumors are extremely rare in pediatric patients and have a very poor prognosis.

Patients who require tricuspid valve surgery constitute a high-risk group. Tricuspid valve repair is associated with better perioperative and long-term outcome than valve replacement. However, patients undergoing replacement showed a significant higher incidence of risk factors for operative mortality. The incidence of re-operation is low with no significant difference when the tricuspid valve has been repaired or replaced. When valve replacement is necessary we recommend the use of a biological prosthesis considering the poor long-term survival.

After the modified Fontan operation, long-term survival in patients with tricuspid atresia was significantly better compared with that in patients with complex congenital malformations. As first-choice therapy for atrial re-entrant tachycardias, we recommend electrophysiologic ablation therapy.

Patients who require TV surgery either as an isolated or a combined procedure constitute a high-risk group. The long-term survival is poor. Tricuspid valve repair with a ring annuloplasty is associated with improved survival and a lower reoperation rate than that with a suture annuloplasty.

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Acute myocardial infarction (MI) is a life-threatening condition rarely encountered in neonates. The patients usually present with sudden cardiogenic shock. Clinical management in neonates is extremely challenging. If treatment is delayed, the prognosis is dismal. We report on a 4-day-old full-term male newborn presenting with acute MI and cardiogenic shock secondary to proximal thromboembolic occlusion of the left descending coronary artery. Hemodynamic stabilization could only be achieved after extracorporeal membrane oxygenation (ECMO) support. Coronary artery patency restoration was performed by selective intracoronary lysis with recombinant tissue plasminogen activator (r-tPA). ECMO support could be discontinued and myocardial function recovered within 6 weeks. We discuss the potential etiologies of acute perinatal MI and the role of ECMO support in the immediate post-MI period. Prompt recognition, timely referral to a cardiac center with availability of specialized advanced treatment options, and management in an orchestrated interdisciplinary approach are crucial for achieving a good outcome.

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