Safety of drug-eluting stents for stenting patent arterial duct in neonates.

OBJECTIVES: The primary objective was to evaluate the trend of blood sirolimus concentrations in neonates following ductal stenting. The long-term outcomes and incidence of infections were also evaluated. METHODS: Prospective open-label observational study in a tertiary referral centre over a 1-year period. Serum sirolimus levels were estimated at 1 hour and 24hrs post-stent insertion followed by 7 days in neonates who underwent ductal stenting. The trend in sirolimus levels, incidence of infections, complications and outcomes following ductal stenting were studied. RESULTS: Seven neonates with duct-dependent pulmonary circulation underwent ductal stenting at median age of 8.5 days and weight of 2.83kg. The average stent size was 3.5±0.4 mm, and average stent length was 16.3±5.1 mm. The mean sirolimus concentrations at 1 hour, 24 hours and 7 days were 41.3±6.9ng/ml, 15.4±7.1ng/ml and 3.1±0.85ng/ml respectively. Levels fell below therapeutic range for all patients by 7 days. Three patients had sepsis or necrotising enterocolitis, but responded well to antibiotics; 1 patient had aspiration related sudden death. There were no further events at a mean follow-up of 207 days, and 4 patients underwent elective surgery at 238 ± 81 days after ductal stenting. CONCLUSIONS: This study demonstrates applicability of drug-eluting stents for ductal stenting in newborns. Drug-eluting stents with abluminal drug delivery are associated with high sirolimus levels in initial hours but rapidly taper to negligible levels within a week of implantation. Neonates with high pre-procedure likelihood of infection developed sepsis but responded well to conservative management. The patency of drug-eluting ductal stents is preserved over long-term follow-up.

Postoperative Aortic Regurgitation in Outflow Ventricular Septal Defect: Determinants of Outcome.

Aortic cusp prolapse is an acquired complication and usually precedes the development of aortic regurgitation (AR) in unoperated outflow ventricular septal defect (VSD). However, its impact on postoperative AR-progression is unknown. 161 patients with outflow-VSD and AR who underwent surgery between 2006 and 2012 were studied retrospectively. 31 patients without prolapse (group-I), 87 with only right coronary cusp (RCC) (group-II), 43 with noncoronary cusp (NCC) prolapse (group-III: 23 only NCC (IIIa), 20 both NCC-RCC (IIIb)) were followed postoperatively for a mean 6.05 ± 2.4 years (range 3-12 years). Moderate or severe-AR was present in 4.2%, 36.8%, 52.2% and 80% preoperatively; in 3.2%, 10.3%, 39.1% and 30% patients at follow-up in group-I, II, IIIa, and IIIb, respectively. Although freedom from significant-AR (moderate or severe AR) or aortic valve replacement (AVR) at 10 years was lesser in subaortic-VSD than subpulmonic-VSD (64.3 ± 7.5% vs 87.9 ± 3.6%; P = 0.02), the difference was not significant when compared within prolapse groups (80 ± 8% vs 88.7 ± 4.0%, P = 0.28 in group-II; 40.7 ± 11.8 vs 70 ± 14.5%, P = 0.48 in group-III). The significant-AR or AVR free survival in patients with trivial or mild preoperative-AR was not significantly different between prolapse groups (98.2 ± 1.8% vs 75 ± 21.7% in group-II and III respectively; P = 0.85). However, in those with moderate or severe preoperative-AR it was significantly lesser in group-III than II (30.1 ± 9.8% vs 65.6 ± 8.4%, respectively; P = 0.04). Group-III, compare to group-II, had 3.28 and 5.24-time risk of development of significant-AR or requirement of AVR, respectively. Prolapse of NCC alone or in addition to RCC prolapse has unfavourable impact on the postoperative outcomes, especially in subaortic-VSD after development of more than mild AR preoperatively.

Outflow Ventricular Septal Defect With Aortic Regurgitation: Optimal Timing of Surgery?

BACKGROUND: Ideal time of surgery still remains controversial in outflow ventricular septal defect (VSD) with aortic regurgitation (AR). We aimed to identify the prevalence and predictors of postoperative AR progression. METHODS: A total of 154 patients with outflow VSD and AR who underwent VSD surgery between 2006 and 2012 were studied retrospectively. RESULTS: Eighty patients with subpulmonic VSD and 74 with subaortic VSD were followed up for mean 6.32 ± 2.27 years (range, 3-12 years). Of these, 100 had trivial to mild (group A) and 54 had moderate to severe preoperative AR (group B). At follow-up, there was no significant worsening of mean residual AR grade in group A (P = .16) and subpulmonic VSD of group B (P = .083). However, AR grade worsened significantly in subaortic VSD (1.85 ± 0.87 vs 2.21 ± 1.08, P = .005) of group B. Only 2 (both had subaortic VSD) patients of group A developed moderate AR and none required aortic valve replacement (AVR), while 23 (42.60%) of group B patients developed moderate or severe AR and 7 (30.4%) of them required AVR. Moreover, all who needed AVR had subaortic VSD and had undergone valvuloplasty during VSD closure. The 10 years freedom from moderate or severe AR was significantly lower in group B than group A in both VSDs (subaortic VSD 42.5% ± 10.7% vs 89.3% ± 8.1%, P < .01; subpulmonic VSD 66.7% ± 10.3% vs 100%, P< .01). On multiple regression analysis, postoperative residual AR was the only predictor of AR progression (standardized coefficient, 0.48; P < .001) at follow-up. CONCLUSIONS: Mild preoperative AR rarely progressed after VSD repair. However, worsening of AR could not be prevented effectively, even with valvuloplasty, after the development of moderate or severe AR. Mild or more postoperative residual AR requires close follow-up, especially in subaortic VSD.

Venous flow patterns after percutaneous atrial septal defect closure: Does the mechanical device decrease right atrial compliance?

INTRODUCTION: Systemic venous flow patterns become abnormal and restrictive after surgical closure of ostium secundum atrial septal defect (ASD) but rarely studied after percutaneous device closure. METHODS: From January 2017 to January 2018, systemic venous Doppler flow patterns were documented prospectively in 50 subjects who underwent percutaneous closure of ASD, prior to, after procedure, and at 6-month follow-up and correlated with defect size and device size. RESULTS: In hepatic veins and superior venacava post device-closure closure, the velocity time integral (VTI) of forward flow in both systole (S) and diastole (D) increased. Overall S was higher than D, and D/S ratio was <1. The D/S ratio increased after device closure significantly reflecting that the improvement in atrial filling increase in diastolic flow more than the increase in systolic flow. Increase in flow velocities was more prominent at 6 months with further increase in D/S VTI ratios. When correlated with the defect size, in those with defect size less than 15 mm/sq.m (mean device size 13.05 ± 3.21 mm), the changes in S- or D-wave, D/S ratio were less prominent and statistically not significant, while in subjects with defect size ≥ 15 mm/sq.m (mean device size 23.02 (±4.77 mm), these changes were greater and statistical significant. CONCLUSION: Residual filling defects with restriction of systolic venous flow were observed in subjects after device closure, correlating with larger device sizes, implying the compliance abnormality conferred by them which progresses at 6 months. Subjects with persistent abnormalities would need careful follow up for incomplete remodeling and increase in atrial size related arrhythmias.

Natural history of aortic valve disease following intervention for rheumatic mitral valve disease.

BACKGROUND AND AIM OF THE STUDY: A significant proportion of patients who require interventions for rheumatic mitral valve (MV) disease have coexisting aortic valve (AV) disease. To date, little is known of the natural history of AV disease in these patients. METHODS: The details of a cohort of 200 patients (146 females, 54 males; mean age at MV intervention 30.3 +/- 9.9 years) with rheumatic heart disease were retrospectively reviewed. The patients had undergone an index MV intervention (either closed or balloon mitral valvotomy) or MV replacement between 1994 and 1996, and received long-term regular follow up examinations. The clinical and echocardiographic data at entry and at follow up were noted. Patients were allocated to two groups, based on whether the AV disease was absent (group I, n=98) or present (group II, n=102) at baseline. The AV disease was categorized as thickening only (group IIA), isolated aortic regurgitation (AR) (group IIB), or combined aortic stenosis (AS) and AR (group IIC). No patient had isolated AS at baseline. RESULTS: The mean follow up period was 9.3 +/- 1.07 years; during which 10 patients in group I developed new AV disease, which included AV thickening only (n=2), trivial-mild AR (n=7) and mild AS with trivial AR (n=1). Of 16 patients in group IIA, 11 developed isolated AR, and one patient progressed to have mild AS and AR. Among 69 patients in group IIB, 22 (31.9%) developed AS, and all had either mild (n=8) or moderate (n=14) AR with mild AS. Group IIC included 17 patients with mild combined AV disease at baseline, except for moderate AS and moderate AR in one patient each. Among 16 patients with mild AS in group IIC, six progressed to moderate AS and two to severe AS. AR became moderate in 10 patients and severe in one patient. The two patients who progressed to severe AS requiring AV replacement had mild AS at baseline. No patient who developed new combined AV disease had lesions with severity more than mild AS or moderate AR. On logistic regression analysis of the variables predisposing to progression of AV disease, such as age, gender, history of rheumatic fever (RF) and recurrence, and interval from RF episode to symptom onset, only the initial AV gradient was identified as being statistically significant (beta coefficient 0.528, SE = 0.17, p < 0.0001). CONCLUSION: Patients with no or mild AV disease at the time of MV intervention rarely develop severe AV disease, and seldom require AV surgery over the long-term follow up. The presence of mild AS at baseline is predictive in the minority of cases where AV disease will progress relatively more rapidly.

Unusual coarctation-the PHACE syndrome: report of three cases.

OBJECTIVES: To report the clinical features, nature of vasculopathy observed and the management instituted in three cases of PHACE syndrome. RESULTS: All three patients were noted to have diffuse vasculopathy and aortic arch hypoplasia in addition to aortic coarctation adding substantial surgical risk or making surgery palliative. CONCLUSION: PHACE syndrome should be considered in the differential diagnosis of aortic coarctation associated with aortic arch hypoplasia.

Hutchinson-Gilford progeria syndrome with severe calcific aortic valve stenosis and calcific mitral valve.

The case of a 12-year-old girl with clinical features of progeria with severe calcific valvar aortic stenosis is presented. The mitral valve showed the presence of calcium, and peripheral vascular disease was also present, though there was no family history of this. Aortic valve replacement was deferred because of insufficient data relating to this condition. The genetics and phenotypic mechanisms of the disease are reviewed. In view of the association of progeria with valve disease, all patients should undergo electrocardiography and echocardiography as part of their routine work-up.