OBJECTIVES:
Our unit has pursued Fontan completion in all patients except those with immobility or combined poor ventricular function and high pulmonary artery pressures. We assessed retrospectively whether conventional high-risk criteria would predict patients with a poorer outcome.
METHODS:
One hundred and thirty-three consecutive children who underwent extracardiac Fontan completion (2004–2012) had their outcomes recorded (mean follow-up of 7 years). Three groups were analysed: those with 1 of 6 historical risk factors (outside 6 commandments), those with 1 of reduced systemic ventricular function or pulmonary artery pressure >15 mmHg (outside 2 commandments) versus those with no contraindications. The Fischer’s exact test examined frequency differences, with the χ2 test to look for outcome associations.
RESULTS:
There were no differences in postoperative complication rates between the outside 6 commandments (n = 105) or outside 2 commandments (n = 49) versus the low-risk no-contraindication group (n = 28): arrhythmias [18% (P = 0.3) or 18% (P = 0.3) vs 25%], infection [22% (P = 0.6) or 33% (P = 0.2) vs 21%], cerebrovascular accident [6% (P = 0.5) or 10% (P = 0.3) vs 4%], length of stay [20 days (P = 0.4) or 23 days (P = 0.2) vs 21 days] and duration of chest drainage (P = 0.5). There was 1 predischarge mortality in each group. Long term, the majority of patients in each group had suitable haemodynamics for fenestration closure [95% (P = 0.7) or 95% (P = 0.7) vs 92%]. Long term, there was no difference in the rate of arrhythmias [11% (P = 0.5) or 12.5% (P = 0.3) vs 7%], protein-losing enteropathy [1% (P = 0.1) or 2% (P = 0.3) vs 7%] or moderate or more ventricular dysfunction on echocardiography [2% (P = 0.7) or 4% (P = 0.7) vs 4%]. Notably, there was a higher rate of catheter reinterventions in the high-risk groups [22% (P < 0.05) or 24% (P < 0.05) vs 7%].
CONCLUSIONS
The medium-term benefits of Fontan completion can be achieved for high-risk patients, suggesting that historical selection criteria should be re-examined.
Isolated AVR in patients aged ≥85 years can be performed with acceptable risk. Survivors improve in NYHA class and, when compared to age- and gender-matched individuals, show a similar life expectancy and a no lower QoL.
Short- and long-term survival following partial AVSD repair in Ireland revealed excellent results compared with other published series. Reoperation incidence also compared excellently with other reports published in the literature.
OBJECTIVE
Our goal was to present 2 decades of our experience with the Ross procedure and its sequential modifications, adopted since 2010, to improve the reoperation rate.
METHODS
We performed a single-centre, retrospective review of database information and medical notes about the implantation technique: the freestanding root. We compared era 1 (1997–2009) and era 2 (2010–2019).
RESULTS
Between 1997 and 2019, a total of 214 Ross procedures were performed (71% men, median age 24 years) [interquartile range (IQR) 15–38]. Of these, 87% had various forms of congenital-dysplastic aortic valves. The median cross-clamping and bypass times were 173 (IQR 148–202) and 202 (IQR 182–244) min. The median postoperative stay was 6 days (2–77). Thirty-day mortality was 0.5%. The median follow-up time was 8.2 years (IQR 3.9–13.2). Survival at 10 and 20 years was 97% and 95%; freedom from greater than moderate aortic regurgitation or aortic valve intervention was 91% and 80%; and 93% of the patients were in New York Heart Association functional class I. Twenty (21%) patients operated on during era 1 and 6 (9%) during era 2 underwent autograft reoperations. The median follow-up time was 14.3 (IQR 11.5–17.4) and 4.8 (IQR 2.5–7) years. Freedom from autograft reoperation was 87% and 69% at 10 and 20 years, with no significant difference between eras. Freedom from homograft reoperation was 96% and 76% at 10 and 20 years. The presence of aortic regurgitation, infective endocarditis and era 1 were predictors of autograft reoperation. Male gender and era 1 were predictors of neoaortic root dilatation.
CONCLUSIONS
The contemporary modified Ross procedure continues to deliver excellent results and should remain part of the strategy to treat children and young adults requiring aortic valve replacement.
Congenital pulmonary airway malformation (CPAM), previously known as congenital cystic adenomatoid malformation (CCAM), is a rare developmental lung abnormality with the potential for malignant transformation. Bronchioloalveolar carcinoma (BAC), pleuropulmonary blastoma (PPB), rhabdomyomatous dysplasia/rhabdomyosarcoma (RMS) have been associated with CPAM. We report an unusual case of a 1-day-old male newborn who underwent lobectomy for a cystic lung lesion, which was found to be a mucinous BAC with K-ras mutation in a type 1 CPAM. The case supports the relationship between type 1 CPAM and BAC/KRAS mutant, and highlights that the malignant transformation can occur in very early stage of the infancy.
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