Long-term follow-up and outcomes of discrete subaortic stenosis resection in children.

BACKGROUND: Studies of long-term outcomes of discrete subaortic stenosis (DSS) are rare. Therefore, we reviewed the long-term outcomes of subaortic membrane resection in children with isolated DSS over 16 years from a single institution. MATERIALS AND METHODS: We retrospectively reviewed the records of patients (n = 27) who underwent resection of DSS between 2000 and 2017. Patients with major concomitant intracardiac anomalies were excluded. Indications for surgery were mean left ventricular outflow tract (LVOT), Doppler gradient >30 mmHg, and/or progressive aortic insufficiency. RESULTS: The mean age at diagnosis was 3.77 ± 3.49 years (range, 0.25-13 years) and the mean age at surgery was 6.36 ± 3.69 years (range, 1-13 years). All patients underwent resection of subaortic membrane. The mean LVOT Doppler gradient decreased from 40.52 ± 11.41 mmHg preoperatively to 8.48 ± 5.06 mmHg postoperatively (P < 0.001). The peak instantaneous LVOT Doppler gradient decreased from 75.41 ± 15.22 mmHg preoperatively to 18.11 ± 11.44 mmHg postoperatively (P < 0.001). At the latest follow-up, the peak gradient was 17.63 ± 8.93 mmHg. The mean follow-up was 7.47 ± 3.53 years (median 6.33 years; range 2.67-16 years). There was no operative mortality or late mortality. Recurrence of subaortic membrane occurred in 7 (25.92%, 7/27) patients who underwent primary DSS operation. Four (14.81%, 4/27) patients required reoperation for DSS recurrence at a median time of 4.8 years (3.1-9.1 years) after the initial repair. Risk factors for reoperation were age <6 years at initial repair. Eighteen (66.66%, 18/27) patients had AI preoperatively and progression of AI occurred in 70.37% (19/27). This included 4 (22.22%, 4/18) patients who had worsening of their preoperative AI. Short valve-to-membrane distance was found to be prognostically unfavorable. One (3.7%, 1/27) patient had an iatrogenic ventricular septal defect, and 2 (7.4%, 2/27) patients had complete AV block following membrane resection. CONCLUSIONS: Resection of subaortic membrane in children is associated with low mortality. Higher LVOT gradient, younger age at initial repair, and shorter valve-to-membrane distance were found to be associated with adverse outcome. Recurrence and reoperation rates are high, and progression of aortic insufficiency following subaortic membrane resection is common. Therefore, these patients warrant close follow-up into adult life.

Transcatheter therapy of partial anomalous pulmonary venous connection with dual drainage and coarctation of the aorta in a single patient.

A 12-year-old boy was found to have aortic coarctation and a partial anomalous pulmonary venous connection. Historically, multiple cardiac pathologies, such as in the present case, required a surgical approach. We describe transcatheter treatment of the coarctation with a stent and occlusion of the partial anomalous pulmonary venous connection with an Amplatzer vascular plug in a single patient without complications.

Q fever endocarditis after right ventricle to pulmonary artery conduit insertion: Case series and review of the literature.

Q fever (QF) is rarely reported in children. Awareness of the disease and newer diagnostic modalities have resulted in increasing recognition of unusual manifestations. We present three cases of QF endocarditis after right ventricle to pulmonary artery conduit insertion in children.

Occlusion of Fontan fenestrations using Amplatzer septal occluder.

The objective of the study is to assess efficacy and safety of the Amplatzer septal device for percutaneous occlusion of Fontan fenestration. Fenestration improves postoperative outcomes in children undergoing Fontan repair. Many of these fenestrations close spontaneously; persistent fenestrations ultimately cause desaturation and can be a potential cause of paradoxical embolism. This is a retrospective review of 26 consecutive patients who underwent transcatheter closure of Fontan fenestration in a tertiary cardiac center in Kuwait. After assessment of hemodynamic suitability for fenestration closure, appropriate balloon sizing of fenestration was obtained. The median age was 9.2 years (range = 1.5-18.3 years). Occlusion was accomplished using a 4-7 ml single Amplatzer septal device in 12 patients and 8-13 ml devices in the other 13 patients; 2 devices were deployed in one patient. Median fluoroscopic and procedure times were 22 and 143.5 min, respectively. One immediate complication was the embolization and successful retrieval of the Amplatzer duct occluder. The fenestration was subsequently occluded by Amplatzer septal device. A 100% occlusion rate of fenestration was achieved in both lateral tunnel and extracardiac conduit types of Fontans. Mean (SD) arterial oxygen saturation increased from 84.7% (4.7) to 95.2% (1.7, P < 0.001) 10 min after deployment of the occluder; Fontan mean circuit pressure had a minimal acceptable increment from 12.9 mmHg (3.2) to 14.6 mmHg (2.6, P < 0.001). No complications or device failures were seen during follow up. The Amplatzer septal occluder device is effective in closing both Fontan fenestrations. Longer single or dual anti-platelet therapy may be considered to prevent thrombotic events. Long-term outpatients follow up with transthoracic echocardiographic monitoring for systemic venous congestion or thrombosis is warranted.

Dilated cardiomyopathy in children and adults: what is new?

Dilated cardiomyopathy (DCM) is the most common form of cardiomyopathy and cause of cardiac transplantation in children and young adults; mortality is high among this patient population. However, mortality, clinical course, and illustrative echocardiographic data of DCM in children and adults are not well established. Our objective was to provide a research article of detailed descriptions of the incidence, causes, outcomes, related risk factors, and new echocardiographic criteria of risk of death from DCM. Our results showed that independent risk factors at DCM diagnosis for subsequent death or transplantation in children cohorts were older age, congestive heart failure, lower left ventricular ejection fraction (EF< or =25%), low global strain, significant mitral valve incompetence, pulmonary hypertension, diastolic dysfunction, right ventricular involvement, and cause of DCM (p<0.001 for all). In adults, low ejection fraction (<30-35%), global peak systolic strain <-7.6%, increased EDV, ESV, LBBB, diastolic dysfunction, and left ventricle dyssynchrony were the main independent risk factors for major cardiac events and need for CRT or transplantation (p<0.001 for all). Our conclusions were that in children and adults, DCM is a diverse disorder with outcomes that depend largely on cause, age, heart failure status at presentation, and echocardiographic parameters of the heart (systolic and diastolic function of left ventricle, pulmonary artery pressure, global strain, and valvular function of the mitral valve). This study will present new findings in the diagnostic area.

The impact of interruption of anomalous systemic arterial supply on scimitar syndrome presenting during infancy.

OBJECTIVES: We sought to evaluate the impact of interruption of anomalous systemic arterial supply (ASAS) on clinical course and management outcome of scimitar syndrome (SS) presenting during infancy. BACKGROUND: No systematic study has been reported so far on this subject, although there are conflicting sporadic reports indicating variable effect. METHODS: Out of 23 children diagnosed to have SS during the past 25 years, 16 symptomatic infants had ASAS. After interrupting ASAS (coil embolization -14, surgical ligation -2), they were prospectively followed up to define their clinical course and management outcome. RESULTS: All 16 infants had sizable ASAS and 9 had variable scimitar vein (SV) stenosis. Fifteen (94%) had pulmonary hypertension and significant left to right shunt. Post intervention, there was variable reduction of shunt in 14 and pulmonary artery pressure in 15 cases. All showed varying clinical improvement. One died of septicemia shortly afterwards. Definitive surgery was deferred for optimal results in seven children for a mean period of 8 months (range 1 month to 3 years). Remaining eight children did not require definitive surgery. One among them had closure of stenosed partial SV by Amplatzer duct occluder ("physiologic correction"). Two children on short term and six children on long term follow-up (mean 5.2 years; range 3.3-10.3 years) are doing well. CONCLUSION: Interruption of ASAS helps to avoid or defer definitive surgery for SS during infancy. Therefore, we recommend coil embolization of ASAS as initial palliation, and long term surveillance to assess need for further intervention.

Transcatheter closure of large pulmonary arteriovenous fistula including pulmonary artery to left atrial fistula with Amplatzer septal occluder.

OBJECTIVES: We sought to evaluate the safety and efficacy of Amplatzer septal occluder (ASO) to close very large pulmonary arteriovenous fistula (PAVF). BACKGROUND: Transcatheter coil embolization, the standard treatment for PAVF, has potential complications particularly in patients with very large fistulas. Several recently available devices have been tried effectively, however, they too have limitations. METHODS: During 2004, three patients (aged 17-56 years), diagnosed with large PAVF including one with pulmonary artery (PA) to left atrial (LA) fistula, had interventional closure prospectively using ASO. Following hemodynamic evaluation and angiographic localization of PAVF, the feeding artery (FA) was selectively cannulated with Amplatzer delivery sheath. ASO, with the right atrial (RA) disc diameter equal to or up to 4 mm larger than the maximum diameter of FA, was delivered through it in such a way that the left atrial disc assumed an oblong shape and the right atrial disc retained its designed flat configuration for better anchorage and thrombogenicity. RESULTS: All patients had very large PAVF fed by a single FA (size 12-24 mm), which was closed completely, without complications, using ASO (size 7-16 mm; RA disc diameter 15-26 mm). Their arterial saturation rose from mean 72.3% to 97.3%. Follow up (1.5-3 years) showed disappearance of the radiological shadows and stable arterial saturations (mean 97.7%). CONCLUSIONS: We conclude that, using the new criteria for device size selection and modified technique of implantation, very large PAVFs including PA to LA fistula can be closed safely, effectively and nonsurgically with ASO.