Unusual Outcome of a Right Ventricular Rhabdomyoma in an Infant.

Rhabdomyomas are the most common benign cardiac tumors. They may often be associated with tuberous sclerosis. In many cases, cardiac rhabdomyomas undergo spontaneous regression. Here, we report the unusual case of an infant with a large nonobstructive right ventricular rhabdomyoma at birth and at four months of age, which subsequently caused severe right ventricle outflow tract obstruction at six months of age, prompting surgery to remove the tumor. Close monitoring should be done in infants with large nonobstructive cardiac rhabdomyomas.

Very long-term outcomes of the Carpentier-Edwards Perimount aortic valve in patients aged 50-65 years.

OBJECTIVES: Aortic valve replacement (AVR) using a bioprosthesis remains controversial for patients aged 50-65 years. This cohort study reports the very long-term outcomes of AVR using Carpentier-Edwards Perimount pericardial bioprosthesis in this age group. METHODS: From 1984 to 2008, 522 Carpentier-Edwards Perimount pericardial aortic bioprostheses were implanted in 516 patients aged 50-65 years (mean age, 60 ± 4 years; 19% female). Multiple valve replacements were excluded fro m our cohort. Baseline demographic, perioperative and follow-up data were recorded prospectively. Mean follow-up was 9 ± 6 years, for a total of 4428 valve-years. Follow-up was complete for 97% of patients included. RESULTS: Operative mortality rate was 2%. One hundred and forty-six late deaths occurred for a linearized rate of 3%/valve-year. Actuarial survival rates averaged 73 ± 2, 59 ± 3 and 35 ± 5% after 10, 15 and 20 years of follow-up, respectively. Mortality rate associated with reoperation was 2%. Actuarial freedom from reoperation rates due to structural valve deterioration (SVD) at 10, 15 and 20 years was respectively of 91 ± 2, 76 ± 3 and 50 ± 6%. Competing risk analysis demonstrated an actual risk of explantation secondary to SVD at 20 years of 30 ± 3%. Expected valve durability was 19 years for this age group. Age was not a significant risk factor for SVD in this middle-aged population. CONCLUSIONS: In patients aged 50-65 years undergoing AVR with the Carpentier-Edwards Perimount bioprosthesis, the expected valve durability was 19 years. Age was not a significant risk factor for SVD within this age group. Patient selection and attention to timing of reintervention may be determinants of long-term outcomes.

Very Long-Term Outcomes of the Carpentier-Edwards Perimount Aortic Valve in Patients Aged 60 or Younger.

BACKGROUND: Aortic valve replacement using a bioprosthesis remains controversial for patients younger than 60 years because of missing data on long-term outcomes in this age group. METHODS: From 1984 to 2008, 383 Carpentier-Edwards Perimount pericardial aortic bioprostheses were implanted in 373 patients 60 years or younger (mean age, 51.0 ± 9.2 years; 19% female). Multiple valve replacements were excluded from our cohort. Baseline clinical, perioperative, and follow-up data were recorded prospectively. The mean follow-up was 8.6 ± 5.9 years, for a total of 3,299 valve-years. Follow-up was complete for 95.3% of patients included. RESULTS: Operative mortality rate was 1.3%. Eighty-five late deaths occurred, for a linearized rate of 2.6%/valve-year. Actuarial survival rates averaged 78.1% ± 2.6%, 65.6% ± 3.5%, and 46.8% ± 6.0% after 10, 15, and 20 years of follow-up, respectively. Mortality rate associated with reoperation was 2.3%. Actuarial freedom from reoperation rates attributable to structural valve deterioration at 10, 15, and 20 years were, respectively, 88.3% ± 2.4%, 70.8% ± 4.1%, and 38.1% ± 5.6%. Competing risk analysis demonstrated an actual risk of explantation secondary to structural valve deterioration at 20 years of 41.6% ± 4.1%. Expected valve durability was 17.6 years for this age group. CONCLUSIONS: In selected patients 60 years or younger undergoing aortic valve replacement with the Carpentier-Edwards Perimount bioprosthesis, the expected valve durability was 17.6 years. Reoperation for structural valve deterioration was associated with a low risk of mortality.