Long-term follow-up of subvalvular aortic stenosis in children: a single-centre experience.

OBJECTIVE: The aim of this study is to evaluate clinical and surgical outcomes of children with subaortic stenosis, to determine the risk factors for surgery and reoperation and to compare isolated subaortic stenosis and those concomitant with CHDs. METHODS: The study involved 80 children with subaortic stenosis. The patients were first classified as isolated and CHD group, and the isolated group was further classified as membranous/fibromuscular group. The initial, pre-operative, post-operative and the most recent echocardiographic data, demographic properties and follow-up results of the groups were analysed and compared. The correlation of echocardiographic parameters with surgery and reoperation was evaluated. RESULTS: There was a significant male predominance in all groups. The frequency of the membranous type was higher than the fibromuscular type in the whole and the CHD group. The median time to the first operation was 4.6 years. Thirty-five (43.7%) patients underwent surgery, 5 of 35 (14%) patients required reoperation. The rate of surgery was similar between groups, but reoperation was significantly higher in the isolated group. The gradient was the most important factor for surgery and reoperation in both groups. In the isolated group besides gradient, mitral-aortic separation was the only echocardiographic parameter correlated with surgery and reoperation. CONCLUSION: Reoperation is higher in isolated subaortic stenosis but similar in membranous and fibromuscular types. Early surgery may be beneficial in preventing aortic insufficiency but does not affect the rate of reoperation. Higher initial gradients are associated with adverse outcomes, recurrence and reoperation.

Subvalvular aortic stenosis: a review of current literature.

Subvalvular aortic stenosis (SAS) is one of the common adult congenital heart diseases, with a prevalence of 6.5%. It is usually diagnosed in the first decade of life. Echocardiography is the test of choice to diagnose SAS. Surgical correction is the best treatment modality, and the prognosis is usually excellent. In this review, we describe the pathophysiology, diagnosis, prognosis, and management of SAS with a focus on different pathophysiologic mechanisms, diagnostic approach, and prognosis of the disease by reviewing the current literature.

Trends and Outcomes of Off-label Use of Transcatheter Aortic Valve Replacement: Insights From the NCDR STS/ACC TVT Registry.

Importance: Transcatheter aortic valve replacement (TAVR) was approved by the US Food and Drug Administration for severe aortic stenosis in patients who cannot undergo surgery and for patients at high operative risk. Use of TAVR for off-label indications has not been previously reported. Objective: To evaluate patterns and adverse outcomes of off-label use of TAVR in US clinical practice. Design, Setting and Participants: Patients receiving commercially funded TAVR in the United States are included in the Transcatheter Valve Therapy Registry. A total of 23 847 patients from 328 sites performing TAVR between November 9, 2011, and September 30, 2014, were assessed for this study. Off-label TAVR was defined as TAVR in patients with known bicuspid valve, moderate aortic stenosis, severe mitral regurgitation, severe aortic regurgitation, or subaortic stenosis. Data were linked with the Centers for Medicare & Medicaid Services for 15 397 patients to evaluate 30-day and 1-year outcomes. Exposure: Off-label use of TAVR. Main Outcomes and Measures: Frequency of off-label TAVR use and the association with in-hospital, 30-day, and 1-year adverse outcomes. Results: Among the 23 847 patients in the study (11 876 women and 11 971 men; median age, 84 years [interquartile range, 78-88 years]), off-label TAVR was used in 2272 patients (9.5%). In-hospital mortality was higher among patients receiving off-label TAVR than those receiving on-label TAVR (6.3% vs 4.7%; P < .001), as was 30-day mortality (8.5% vs 6.1%; P < .001) and 1-year mortality (25.6% vs 22.1%; P = .001). Adjusted 30-day mortality was higher in the off-label group (hazard ratio, 1.27; 95% CI, 1.04-1.55; P = .02), while adjusted 1-year mortality was similar in the 2 groups (hazard ratio, 1.11; 95% CI, 0.98-1.25; P = .11). The median rate of off-label TAVR use per hospital was 6.8% (range, 0%-34.7%; interquartile range, 3.4%-12.1%), with hospitals in the highest tertile of off-label use associated with increased 30-day adverse cardiovascular events compared with the lowest tertile. However, this difference was not observed in adjusted 30-day or 1-year outcomes. Conclusions and Relevance: Approximately 1 in 10 patients in the United States have received TAVR for an off-label indication. After adjustment, 1-year mortality was similar in these patients to that in patients who received TAVR for an on-label indication. These results reinforce the need for additional research on the efficacy of off-label TAVR use.

Influencia de la fragilidad del paciente en las enfermedades cardiovasculares / The influence of frailty on outcomes in cardiovascular disease

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Modified single patch: are we still worried about subaortic stenosis?

BACKGROUND: When the modified single-patch technique for atrioventricular septal defect (AVSD) repair was introduced by Dr Benson Wilcox, there was concern that these patients might be at risk for late subaortic stenosis and left ventricular outflow tract obstruction (LVOTO). This review evaluated our modified single-patch population for LVOTO in the postoperative period. METHODS: Between January 2000 and 2013, 77 infants underwent AVSD repair with a modified single-patch technique. Median age was 4.2 months, and median weight was 5 kg. Eight patients had a prior repair of coarctation of the aorta via left thoracotomy in the newborn period. RESULTS: The median hospital stay was 10 days. No patient required a pacemaker. The mean and median follow-up times were 4.6 and 3.7 years, respectively. Only 2 patients (2.5%) required reoperation for LVOTO; both had prior repair of coarctation of the aorta (2 of 8 vs 0 of 69, p = 0.01). A discrete fibrous subaortic membrane developed in the first patient that required resection at 3 and 7 years after repair. The other patient had LVOTO from accessory chordae of the left atrioventricular valve and required mitral valve replacement 5 months after repair. One early death occurred at 4 months postoperatively due to liver failure related to hyperalimentation. CONCLUSIONS: At intermediate term follow-up, LVOTO does not appear to be a significant postoperative issue after modified single-patch repair of AVSD. Coarctation of the aorta was the most significant predictor of late LVOTO after repair of AVSD with the modified single-patch technique.

Evaluation of subvalvular aortic stenosis in children: a 16-year single-center experience.

Subvalvular aortic stenosis accounts for 1-2 % of all congenital heart disease and for 8-20 % of cases of left-ventricular outflow tract (LVOT) obstruction in children. Recurrence of subaortic stenosis (SAS) is not uncommon after surgical management. This study was performed to investigate the clinical and surgical outcomes and to estimate the predictability of recurrences of SAS. Seventy-nine patients age 3-21 years with SAS between 1994 and 2010 were reviewed. Fifty-one patients had discrete SAS, whereas the remaining 15 patients had fibromuscular ridge-type SAS. Mean follow-up time without surgery was 22 months (range of 1-94). Forty-one patients with a diagnosis of SAS underwent surgery. Recurrence rates were 22.7 % (15 patients), and these patients developed SAS at a mean of 4.7 years follow-up. We performed second surgical membrane resection in only 1 patient. The risk of recurrence of SAS was only linked to higher preoperative LVOT gradient. Twenty-three patients had no aortic regurgitation (AR) at preoperative echocardiography. Of these, 39.1 % had trivial, 8.7 % had mild, and 8.7 % had moderate AR after surgery; there was no significant AR. We conclude that surgical intervention was required most of the time in patients with SAS, and surgical outcomes was excellent even if there were associated cardiac defects. The risk of recurrences was higher, especially in patients with higher initial LVOT gradients, although a second surgery was rarely necessary in these patients.

Caregiver survey of pharmacotherapy to treat attention deficit/hyperactivity disorder in individuals with Williams syndrome.

Williams syndrome (WS) is a genetic condition characterized by a unique neurocognitive and behavioral profile, including increased incidence of attention deficit/hyperactivity disorder (ADHD). The purpose of the present study was to examine the perceived helpfulness and side effects of medications used to treat ADHD (methylphenidate class, amphetamine class, atomoxetine) in individuals with WS. This was accomplished with a survey of parents/caregivers of individuals with WS through the Williams Syndrome Association. Five-hundred twelve (512) parents/caregivers responded to the survey regarding their child's/adult child's use of ADHD medications. Twenty-seven percent (27%) of the individuals had been prescribed a medication for ADHD, most commonly a methylphenidate class medication. OROS-methylphenidate was reported as the most helpful methylphenidate class formulation, with 74% reporting it at least somewhat helpful. Survey participants reported similar side effects as typically developing controls, but to a greater degree. Irritability was the most commonly endorsed side effect of an ADHD medication (38%). Individuals reported use of stimulant medications in the presence and absence of underlying cardiac conditions, with 56% of ADHD medication users reporting supravalvular aortic stenosis, 36% pulmonary artery stenosis, and 25% systemic hypertension. Individuals taking ADHD medications were more likely to report dental problems (p=0.004). Additional studies are needed to further investigate these findings and examine short-versus long-acting stimulant medications and dosage effects.

Guía de práctica clínica sobre el tratamiento de las valvulopatías (versión 2012) / Guidelines on the Management of Valvular Heart disease (Version 2012)

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The relevance of echocardiography heart measures for breeding against the risk of subaortic and pulmonic stenosis in Boxer dogs.

The aims of this study were to investigate the role and relative importance of auscultation and echocardiography traits as risk factors for the diagnosis of subaortic (SubAS) and pulmonic (PS) stenosis and to estimate the heritability (h(2)) of cardiac measurements taken through echocardiography for a random sample of Italian Boxer dogs. The data were cardiovascular examination results of 1,283 Italian Boxer dogs (686 females and 597 males) enrolled in the national screening program for heart defects arranged by the Italian Boxer Club. Examinations were performed during a 6-yr period by a group of 7 veterinary cardiologists following a standard protocol. Occurrence and severity of SubAS and PS were diagnosed, taking into account clinical and echocardiography findings such as the grade of cardiac murmur, direct ultrasound imaging of the anatomic obstructive lesions, and values of aortic or pulmonary blood flow velocities. A Bayesian logistic regression analysis was performed to identify clinical and echocardiography variables related to SubAS and PS diagnosis. Estimation of variance components for clinical and echocardiography traits was performed using a mixed linear animal model, Bayesian procedures, and the Gibbs sampler. Prevalence of SubAS (PS) was 8.4% (2.2) and 10.7% (6.4) for female and male dogs, respectively. Cardiac murmur, peak velocities, and annulus areas behaved as risk factors for SubAS and PS. The risk of a positive diagnosis for SubAS was 3 times greater for dogs with aortic annulus area <2.1 cm(2) relative to dogs with areas >2.37 cm(2), 84 times greater for dogs showing aortic peak velocities >2.19 m/s relative to dogs with peak velocities <1.97 m/s, and 41 times greater for dogs with moderate to severe murmur grades relative to dogs with absent murmur. Similar results were obtained for PS. The estimated h(2) for the occurrence of cardiac defects was 23.3% for SubAS and 8.6% for PS. Echocardiography and cardiac murmur grades exhibited moderate h(2) estimates and exploitable additive genetic variation. The estimated h(2) was 36, 24, and 20% for aortic annulus area, aortic peak velocity, and cardiac murmur score, respectively. For the area of the pulmonary annulus and peak pulmonary velocity, the estimated h(2) were smaller, ranging from 9.5 to 12.8%. These measures are candidate indicator traits that might be effectively used in dog breeding to reduce the prevalence and severity of cardiac defects.

Enfermedad cardiovascular en el anciano / Cardiovascular disease in the elderly

El envejecimiento de la población en todo el mundo dará lugar a un número creciente de pacientes ancianos, en los que la cardiopatía es la principal causa de muerte. Las alteraciones de la fisiología cardiovascular con el envejecimiento normal y las comorbilidades causan diferencias en los problemas cardiacos y en la respuesta a los tratamientos en los pacientes ancianos. Los objetivos de la asistencia centrados en el paciente, como el mantenimiento de la independencia y la reducción de los síntomas, pueden ser más prioritarios que el aumento de la longevidad. Es probable que los nuevos tratamientos menos invasivos mejoren los resultados obtenidos en pacientes ancianos en los que antes se consideraba que el riesgo de los procedimientos tradicionales impedía su aplicación. Los ensayos clínicos en los que se ha incluido a pacientes ancianos son limitados y es frecuente que las recomendaciones de tratamiento basadas en pacientes de menor edad carezcan de respaldo en la evidencia para los pacientes de edad superior a 75 años (AU)

The aging of the population worldwide will result in increasing numbers of elderly patients, among whom heart disease is the leading cause of death. Changes in cardiovascular physiology with normal aging and prevalent comorbidities result in differences in the effects of common cardiac problems as well as the response to their treatments. Patient-centered goals of care such as maintenance of independence and reduction of symptoms may be preferred over increased longevity. New less-invasive treatments are likely to improve outcomes in elderly patients who previously have been considered at prohibitive risk for traditional procedures. Clinical trials enrolling elderly patients are limited and recommendations for management from younger patients frequently lack evidence-based support in patients aged >75 years (AU)

Prevalence and associated risk factors for intervention in 313 children with subaortic stenosis.

BACKGROUND: We sought to determine the prevalence of intervention and associated factors in children presenting with subaortic stenosis. We also investigated whether a protocol adopted in 1994 of early subaortic resection at a preoperative mean systolic gradient across the left ventricular outflow tract (LV gradient) greater than 30 mm Hg was supported by longitudinal outcomes. METHODS: Record review of all children (n = 313) diagnosed with subaortic stenosis was conducted between 1975 and 1998 at our institution. Cox proportional hazard models determined the prevalence and associated factors for initial subaortic resection. Mixed models of serially obtained echocardiographic data (n = 933) established longitudinal LV gradient trends and identified factors associated with more rapid LV gradient progression. RESULTS: Median age at presentation was 8 months. Freedom from initial subaortic resection was 40% at 16 years from diagnosis. Earlier progression to subaortic resection was associated with patient characteristics at presentation, including a higher initial LV gradient (p < 0.001), larger aortic annulus z-score (p = 0.005), smaller body surface area (p < 0.001), and smaller mitral annulus z-score (p = 0.003). Initial resection was also associated with a faster rate of LV gradient progression (p = 0.003). Factors determining the increased rate of LV gradient progression included an initial LV gradient greater than 30 mm Hg (p < 0.001), initial aortic valve thickening (p = 0.003), and attachment of subaortic stenosis to the mitral valve (p = 0.003). Worse aortic regurgitation grade with time was also associated with an initial LV gradient greater than 30 mm Hg (p < 0.001). CONCLUSIONS: Subaortic resection should be delayed until the LV gradient exceeds 30 mm Hg because most children with an initial LV gradient less than 30 mm Hg have quiescent disease.

Intramyocardial arterial narrowing in dogs with subaortic stenosis.

Earlier studies have described intramyocardial arterial narrowing based on hyperplasia and hypertrophy of the vessel wall in dogs with subaortic stenosis (SAS). In theory, such changes might increase the risk of sudden death, as they seem to do in heart disease in other species. This retrospective pathological study describes and quantifies intramyocardial arterial narrowing in 44 dogs with naturally occurring SAS and in eight control dogs. The majority of the dogs with SAS died suddenly (n=27); nine had died or been euthanased with signs of heart failure and eight were euthanased without clinical signs. Dogs with SAS had significantly narrower intramyocardial arteries (P<0.001) and more myocardial fibrosis (P<0.001) than control dogs. Male dogs and those with more severe hypertrophy had more vessel narrowing (P=0.02 and P=0.02, respectively), whereas dogs with dilated hearts had slightly less pronounced arterial thickening (P=0.01). Arterial narrowing was not related to age, but fibrosis increased with age (P=0.047). Dogs that died suddenly did not have a greater number of arterial changes than other dogs with SAS. This study suggests that most dogs with SAS have intramyocardial arterial narrowing and that the risk of dying suddenly is not significantly related to the overall degree of vessel obliteration.

St. Jude Medical Toronto biologic aortic root prosthesis: early FDA phase II IDE study results.

BACKGROUND: Several biological aortic root replacement techniques have distinct advantages over mechanical composite root replacement including better valvular hemodynamic characteristics and the lack of need for anticoagulation. Current biological root replacement options lack proven long-term durability or are limited by technical or practical concerns. We report the early results from a phase II multicenter clinical trial of the porcine St. Jude Toronto Bioprosthesis with BiLinx (Toronto root). METHODS: 176 Toronto roots were implanted as total aortic root replacement from August 2001 through August 2003. Concomitant cardiac procedures including coronary artery bypass grafting (31%) and ascending aortic replacement (55%) were performed in 74%. Patients were followed clinically and were examined with an echocardiogram at discharge, 6 months, 12 months, and yearly thereafter. Root sizes implanted included 29 mm in 38%, 27 mm in 30%, 25 mm in 20%, 23 mm in 10%, and 21 mm in 2.2%. RESULTS: There are 205 patient years of follow-up through October 2003. Operative mortality was 3.9% (none were valve related) and late mortality was 4%. Operative stroke rate was 1.1% and late stroke rate was 0.6%. Endocarditis developed in 1 patient. Freedom from aortic regurgitation is to date 100% at discharge, 6 months, and 1 year postimplant. Reoperation of the aortic valve/root was not required in any patient. Six-month mean transvalvular gradients for 21-29 mm valves were 12.8, 8.8, 5.3, 4.9, and 4.7 mm Hg, respectively. CONCLUSIONS: Aortic root replacement with the Toronto root is safe and provides superb transvalvular hemodynamics with freedom from anticoagulation. The Toronto root seems widely applicable for all types of aortic root pathology and these early data offer very encouraging results. Long-term follow-up is required.

Discrete subaortic stenosis: incidence, morphology and surgical impact of associated subaortic anomalies.

BACKGROUND: The association between discrete subaortic stenosis and other subaortic anomalies is a well known but rarely reported occurrence. The aim of this study is to define the incidence, morphology, and surgical impact of associated anomalies of the left ventricular outflow tract in children operated on for discrete subaortic stenosis. METHODS: Between 1994 and 2000, 45 consecutive children were operated on for discrete subaortic stenosis. Patients were divided in two groups according to the obstructive lesion detected by echocardiography. RESULTS: A localized shelf was found as an isolated lesion in 31 patients (group A), whereas additional subaortic anomalies were found in 14 cases (31%) and were multiple in 5 cases (group B). The anomalies included anomalous septal insertion of mitral valve (7 cases); accessory mitral valve tissue (2 cases); anomalous papillary muscle (2 cases); anomalous muscular band (8 cases); and muscularization of the anterior mitral valve leaflet (1 case). Cardiopulmonary bypass and aortic cross-clamping times were significantly shorter in group A. There were no operative deaths nor major complications or deaths during follow-up. A gradient of 15 mm Hg or more was found at follow-up in 5 cases whereas aortic regurgitation was estimated to be not clinically significant in all but 1 patient. Six cases of recurrent subaortic stenosis were found in our series, 3 of them with other subaortic anomalies. CONCLUSIONS: This study shows that discrete subaortic stenosis can often be associated with other subaortic abnormalities. Surgical treatment of these anomalies produces excellent early and mid-term relief of obstruction without any increase in mortality and morbidity.

Subaortic stenosis: at risk substrates and treatment strategies.

Subaortic stenosis can be a complex disease of multiple anatomic etiologies. At the core is either an elongated and narrow outflow tract as compared to normal or a fully muscle-rimmed VSD used as an intraventricular routing pathway. An array of treatment modalities is needed for an effective management strategy.

Benefits of early surgical repair in fixed subaortic stenosis.

OBJECTIVES: We sought to determine whether early resection can improve outcome in fixed subaortic stenosis. BACKGROUND: The diagnosis of subaortic stenosis (SAS) is often made before significant gradients occur. Whereas resection is the accepted treatment, it remains uncertain whether surgical intervention at this early stage can reduce the incidence of recurrence or influence the progression of aortic valve damage. METHODS: Follow-up was available for 75 of 83 consecutive patients operated on for fixed SAS; the average duration of follow-up was 6.7 years. The lesion was discrete in 68 patients (91%) and of a tunnel type in 7, with associated ventricular septal defect in 28 (37%). All underwent transaortic resection. RESULTS: There were no deaths. There were 18 recurrences of SAS in 15 patients (20%). Thirteen patients (17%) underwent 17 reoperations for recurrence or aortic valve disease. The cumulative hazard of recurrence was 8.9%, 16.1% and 29.4% +/- 2.3% (mean +/- SEM), and the hazard of events, including recurrence and reoperation, was 9.2%, 18.4% and 35.1% +/- 3.5% at 2, 5 and 10 years, respectively. Residual end-operative left ventricular outflow tract (LVOT) gradients (> 10 mm Hg, n = 8) and tunnel lesions were univariate predictors of recurrence (p = 0.0006 and p = 0.003, respectively). Multivariate predictors included higher preoperative LVOT gradient (p < 10(-4)) and younger patient age (p = 0.002). Only two recurrences (0.87 per 100 patient-years of follow-up) were noted in patients with a preoperative peak LVOT gradient < or = 40 mm Hg (n = 40), whereas higher gradients (n = 35) were associated with a greater than sevenfold recurrence rate (6.45 events per 100 patient-years, p = 0.002). The aortic valve required concomitant repair in 17 cases in the high gradient group (48.6%) but in only 8 in the low gradient group (20%, p = 0.018). Despite relief of the obstruction, progressive aortic regurgitation was noted at follow-up after 14 procedures in the high gradient group (40%) but after only 5 procedures in the low gradient group (12.5%, p = 0.014). CONCLUSIONS: The data suggest that surgical resection of fixed subaortic stenosis before the development of a significant (> 40 mm Hg) outflow tract gradient may prevent recurrence, reoperation and secondary progressive aortic valve disease.

Hpertrophic subaortic stenosis is not rare in the eighth decade.

During a five year period, idiopathic hypertrophic subaortic stenosis was diagnosed in nine patients 70 to 79 years of age and in 26 patients 20 to 66 years of age who were referred to the cardiology division of the Long Island Jewish-Hillside Medical Center because of symptomatic heart disease. Only one of the older patients, compared with 19 of the younger ones, was referred with the correct diagnosis. Coronary artery disease with papillary muscle dysfunction was incorrectly diagnosed in four of the older patients. Cardiac catheterization confirmed the diagnosis of idiopathic hypertrophic subaortic stenosis in all of the 26 younger patients and in five of the nine older ones; diagnosis was based on typical echocardiographic features in the other four patients. Symptoms, physical findings, and electrocardiographic observations were similar in both groups. Aortic valve calcification was found in one younger patient and three older ones. The ratio of women to men was higher in the older group (7:2) than in the younger group (12:14). Coronary artery disease was more frequent in the older patients (three of five, compared with six of 26). Our experience indicates that idiopathic hypertrophic subaortic stenosis is not rare during the eighth decade. The diagnosis should be considered in any patient with an unexplained appropriate heart murmur, and definitive studies, such as echocardiography, should be done. As with any condition, proper management depends on accurate diagnosis.