Konno-Rastan Operation in a Patient With Dextrocardia and Situs Inversus.

The Konno-Rastan operation is performed for relief of complex left ventricular outflow obstruction with a small aortic annulus. When associated with situs inversus and dextrocardia, important aspects should be kept in mind due to the mirror-image anatomy. In this report, we present a case of a 10-year-old child with the diagnosis of recurrent diffuse subaortic stenosis and situs inversus and dextrocardia who underwent the Konno-Rastan operation successfully and was asymptomatic with normal physical activity after a follow-up period of one year.

Association of sotalol versus atenolol therapy with survival in dogs with severe subaortic stenosis.

INTRODUCTION/OBJECTIVES: Dogs with severe subaortic stenosis (SAS) are at risk of dying suddenly from fatal arrhythmias. Survival is not improved when treated with pure beta-adrenergic receptor (ß)-blockers; however, the effect of other antiarrhythmic drugs on survival is unknown. Sotalol is both a ß-blocker and a class III antiarrhythmic drug; the combination of these differing mechanisms may provide benefit to dogs with severe SAS. The primary objective of this study was to compare survival in dogs with severe SAS that were treated with either sotalol or atenolol. The secondary objective was to evaluate the effect of pressure gradient (PG), age, breed, and aortic regurgitation on survival. ANIMALS: Forty-three client-owned dogs. MATERIALS AND METHODS: Retrospective cohort study. Medical records of dogs diagnosed with severe SAS (PG ≥ 80 mmHg) between 2003 and 2020 were reviewed. RESULTS: No statistical difference was identified in survival time between dogs treated with sotalol (n=14) and those treated with atenolol (n=29) when evaluating all-cause mortality (p=0.172) or cardiac-related mortality (p=0.157). Of the dogs that died suddenly, survival time was significantly shorter in dogs treated with sotalol compared to those treated with atenolol (p=0.046). Multivariable analysis showed that PG (p=0.002) and treatment with sotalol (p=0.050) negatively influenced survival in the dogs that died suddenly. CONCLUSIONS: Sotalol did not have a significant effect on survival overall but may increase the risk of sudden death in dogs with severe SAS compared to atenolol.

"(Pseudo)cavities inside the heart: What may they unfold?"

Subvalvular aortic stenosis manifesting as a subaortic membrane predisposes to bacterial endocarditis, which typically affects the aortic valve (AoV) or, less frequently, the left ventricular outflow tract (LVOT). We present the case of a 60-year-old woman expressing an odd form of a subvalvular aortic membrane in conjunction with a left Valsalva sinus pseudoaneurysm as a result of an endocarditis complication.

Bicuspid aortic valve caused subaortic stenosis with bulging of valve calcification through subaortic area in a young patient.

Bicuspid aortic valve is the most common congenital cause for the development of aortic valve calcification and stenosis. Calcification cause valvular stenosis or valvular insufficiency due to coaptation failure. We report a unique case of calcification of bicuspid valve was extending to left ventricular outflow tract and attached to interventricular septum which caused subvalvular stenosis.

[Epidemiological, clinical and echographic profile of patients operated for subvalvular aortic stenosis in the region of Sfax (Tunisia) and factors associated with postoperative recurrence: an observational study]. / Profil épidémiologique, clinique et échographique des patients opérés pour un rétrécissement aortique sous-valvulaire dans la région de Sfax (Tunisie) et facteurs associés à la récidive post-opératoire: étude observationnelle.

Subvalvular aortic stenosis is difficult to manage due to the evolutionary unpredictability of stenosis and a high recurrence rate after surgical treatment. The purpose of this study is to describe the profile of patients undergoing surgery for the treatment of aortic subvalvular stenosis and to investigate factors associated with post-operative recurrence of the subaortic obstacle. We conducted an observational study of all patients operated for subvalvular aortic stenosis, whose data were collected in the Department of Cardiology of the Sfax University Hospital between January 2010 and December 2020. The study involved 28 patients, with predominance of male sex (64.29%, n=18). At diagnosis, the mean age was 6.82 (±4.84) years and 19 patients (67.85%) had symptoms. On echocardiography, maximal subaortic gradient ≥50 mmHg was found in 23 patients (82.14%). Cardiovascular malformations associated with subvalvular stenosis were found in 16 patients (57.14%). The average age of patients at the time of surgery was 10.43 (±7.08) years. Subaortic membrane resection was the most commonly used technique (46.4%, n=13). It was associated with septal myomectomy in 8 patients (28.6%). Postoperative mortality rate was zero. Residual gradient ≥30 mmHg was reported in 8 patients (28.6%) after surgery. Recurrences were observed in 7 patients (25%) of whom 6 underwent reintervention. In multivariate analysis, only postoperative residual gradient was significantly associated with recurrence (p=0.030, OR=33.785, 95% CI: 1.398-816.754). Despite old age at diagnosis and surgery, favorable short-term outcomes were reported, but recurrences were frequent in the long term. This highlights the role of regular, perioperative and postoperative, clinical and echographic monitoring of these patients.

Echocardiographic and electrocardiographic effects of atenolol versus sotalol in dogs with severe subaortic stenosis.

BACKGROUND: Subaortic stenosis (SAS) is a commonly diagnosed canine congenital cardiac defect, with severe forms of carrying a poor long-term prognosis. To date, an effective treatment strategy has not been developed in veterinary medicine. This study sought to determine if sotalol, a class III antiarrhythmic, may have salient echocardiographic and antiarrhythmic benefits for medical management for dogs affected with severe SAS. METHODS: Ten dogs diagnosed with severe SAS were enrolled in this prospective, double-blinded, crossover study. Dogs underwent physical exam, non-invasive blood pressure measurement, electrocardiography, echocardiography, and 24-h Holter monitoring. Diagnostics were repeated 12-16 days following randomization to oral atenolol (0.5-1 mg/kg) or sotalol (1-2 mg/kg) twice daily. After a medication taper and four-day washout, dogs were crossed-over to the alternate study medication, and the diagnostics were repeated in 12-16 days. Linear and multinomial mixed models were developed to evaluate the effects of treatments on echocardiographic and electrocardiographic variables. RESULTS: Indices of left ventricular systolic function were reduced based on the volumetric assessment when dogs received sotalol compared to atenolol. No difference was noted between groups in left ventricular systolic function based on the linear assessment. No difference was observed in the reduction in left ventricular outflow tract velocity. No significant differences were observed between treatment groups for any variable on 24-h Holter monitor. CONCLUSIONS: Sotalol may be a viable therapy to consider for dogs with severe SAS based on this pilot study. A larger, prospective study is necessary to investigate further.

Avaliação da prática esportiva em atleta jovem com estenose aórtica subvalvar corrigida / Evaluation of sports practice in a young athlete with corrected subvalvular aortic stenosis

Paciente 18 anos, sexo masculino, sem outras comorbidades, ex-atleta amador praticante de Rugby, interrompeu a prática esportiva por orientação médica após o diagnóstico de estenose subvalvar aórtica grave, com gradiente médio de 81 mmHg e máximo de 158 mmHg em membrana subvalvar. Foi realizada correção cirúrgica com ressecção da membrana subaórtica associada à ressecção muscular da via de saída do ventrículo esquerdo e plastia da valva tricúspide. Após recuperação cirúrgica paciente, mantém-se assintomático e retorna periodicamente para avaliação quanto a prática de atividade física e esportiva competitiva. Três anos após cirurgia, em avaliação complementar, ao ecocardiograma, observava-se persistência de prega ventrículo-infundibular e resquício de membrana, determinando estreitamento da via de saída do ventrículo esquerdo (VE), com aceleração de fluxo gerando gradiente sistólico máximo entre VE e aorta de 42 mmHg e médio de 24 mmHg. Refluxo aórtico discreto a moderado. Função sistólica biventricular preservada, hipertrofia ventricular esquerda discreta. Foi solicitado também ecocardiograma com estresse físico que não mostrou sinais de isquemia miocárdica. Membrana subaórtica gerou obstrução importante com repercussão hemodinâmica ao esforço físico. Por fim, realizou teste ergométrico, que apresentou Infradesnivelamento de segmento ST de 2,5mm horizontam em MC5, sem dor, com comportamento adequado da pressão e da frequência cardíaca durante o esforço e capacidade funcional estimada de 13 METs. Após exames complementares, foi realizado reunião com especialistas em cardiologia do esporte e liberado para a prática de atividade física moderada, com cálculo de frequência máxima até 60% da FC reserva e resistiva com até 50% da força máxima. A estenose subvalvar aórtica é uma cardiopatia congênita relativamente frequente que resulta de uma anormalidade subjacente na arquitetura da via de saída do ventrículo esquerdo (VSVE), com fluxo turbilhonado levando à fibrose progressiva dessa região. Obstrução significativa da VSVE leva à hipertrofia ventricular e função hiperdinâmica. É a segunda causa mais comum de estenose aórtica na população pediátrica e muitos pacientes têm regurgitação aórtica associada. A correção cirúrgica geralmente é indicada a partir da 2ª década de vida e 20 até 30% dos pacientes evoluem com recorrência da obstrução. Em virtude da prevalência dessa condição em indivíduos jovens, além de outras condições de risco, a avaliação médica por especialista antes da prática esportiva competitiva torna-se essencial.

Surgical Repair of Secondary Subaortic Stenosis in Congenital Heart Disease Without Initial Subaortic Obstruction.

BACKGROUND: Secondary subaortic stenosis (SSS) is a rare heart disease of the left ventricular outflow tract (LVOT). It usually occurs after cardiovascular correction with or without initial left ventricular outflow tract obstruction (LVOTO). Because most patients with SSS are asymptomatic, many do not realize the need for reoperation until the obstruction worsens. Few studies suggest the characteristics and reasons of SSS without initial SAS. We conducted a retrospective study to describe the characteristics and surgical outcomes of these patients. METHODS: In this study, we examined a single-center retrospective cohort of SSS patients without initial SAS undergoing resection from 2010 to 2019. Patients are defined as secondary subaortic obstruction requiring surgery after cardiovascular correction. Demographics, perioperative findings, and clinical data were analyzed. RESULTS: Twenty-three patients had undergone secondary cardiac surgery for SSS without initial SAS during 10 years in our center. The median age at operation was 7.3 (4.0-13.5) years. In this study, the most commonly associated cardiac lesions were ventricular septal defect (VSD), atrioventricular septal defect (AVSD), patent ductus arteriosus (PDA), and coarctation arch hypoplasia (COA). The surgical techniques included membranous resection of five patients, fibromuscular resection of 17 patients, and reconstruction of the intraventricular baffle of one patient. The results of surgery in these patients are satisfied. The average LVOT gradient at the last follow up was 14.9 (7.8-26.2) mmHg. There was no operative mortality. Two patients had postoperative complications. The median follow-up period was 2.9 (1.1-4.3) years with one late death. Two patients (8.7%) had recurrence of stenosis. CONCLUSIONS: Secondary subaortic stenosis is an uncommon heart disease. The reason is related to several causes, including missed diagnosis, unnoticed abnormalities of LVOT, and further changes of geometric morphology by intracardiac surgery. The results of surgery in these patients are satisfied. However, the recurrence of stenosis is still frequent.

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.

Natural history of subaortic stenosis in 166 dogs (1999-2011).

INTRODUCTION: Subaortic stenosis (SAS) is one of the most common congenital cardiac diseases in dogs. The objective of this study was to provide survival times on a large population of dogs with SAS and to propose a redefined pressure gradient (PG) scale to include a mild, moderate, severe and very severe disease group. ANIMALS, MATERIALS AND METHODS: Dogs were divided into four groups based on the Doppler-derived PG across the stenosis. Disease severity was defined as follows: mild = PG < 50 mmHg; moderate = PG range 50-80 mmHg; severe = PG range 80-130 mmHg; and very severe = PG > 130 mmHg. Over the study period (1999-2011), 166 client-owned dogs were diagnosed with SAS of which 129 had follow-up information available. RESULTS: Unadjusted median survival time for severity groups were as follows: mild 10.6 years; moderate 9.9 years; severe 7.3 years; and very severe 3.0 years. Univariable analysis examining the effect of the PG, age at diagnosis and sex found only the PG and age at diagnosis had a significant effect on survival. Adjusted survival curves showed that the survival time in the very severe group was decreased compared with all other groups. CONCLUSION: Based on the results of this study, a revised SAS classification system with four PG groups is appropriate. Dogs with a PG > 130 mmHg were identified as those with the lowest median survival time.

Surgical repair of subaortic stenosis resection: 10 years of single-center experience in 65 patients.

BACKGROUND: Subaortic stenosis (SAS) was a rare congenital heart disease of left ventricular outflow tract (LVOT), ranging from "isolated" lesions to "tunnel" or "diffuse" lesions. We conducted a retrospective study to describe the characteristics of patients with different lesions and analyze the risk factors for reoperation. METHODS: In this study, we examined a single-center retrospective cohort of SAS patients undergoing resection from 2010 to 2019. Patients were classified as simple lesion group (n = 37) or complex lesion group (n = 28). Demographics, perioperative findings, and clinical data were analyzed. RESULTS: The surgical effect of the two groups was significantly lower than that before the operation (p < .05). The median age at operation was 6 (3-11.8) years. There was no operative mortality. In complex lesion group, cardiopulmonary bypass time (CPB time), aortic cross-clamping time (ACC time), mechanical ventilation time, and intensive care unit (ICU) stay time were longer. The median follow-up period was 2.8 years (range: 1-3.8), with two late death. Six patients (9.2%) required reoperation due to restenosis or severe aortic insufficiency. The freedom from reoperation rates at 5 years was 66.7% for simple lesion but only 52.3% for complex lesion (p = .036). CONCLUSIONS: Although the lesions include many forms, SAS resection was still satisfactory. However, the reoperation after initial surgical treatment was not infrequent, especially in patients with complex lesion.

Membrana subvalvar aórtica em adulto, diagnóstico por angiotomografia: relato de caso / Aortic subvalvular membrane in an adult, diagnosed by CT angiography: case report

INTRODUÇÃO: A angiotomografia computadorizada (AngioTC) tem mostrado seu papel de impacto no diagnóstico de cardiopatias congênitas (CC), por ser de ótima acurácia e não invasiva. A Estenose Aórtica Subvalvar (EAoS), uma CC, rara, que pode ser causa de obstrução à via de saída do ventrículo esquerdo (VSVE). CASO CLÍNICO: Homem,79 anos, hipertenso, com quadro de angina CCS III e síncope. Ao exame físico: sopro sistólico em foco aórtico +3/+6, irradiado para fúrcula esternal. Ecocardiograma transtorácico (ECOTT): Fração de ejeção 69%, valva aórtica trivalvular(VAo), espessadas e calcificadas, refluxo discreto, área valvar de 1,0cm2, gradiente sistólico médio 49mmHg. Devido à estenose aórtica (EAo) estágio D1, de risco intermediário, foi indicado o implante transcateter da válvula aórtica (TAVI) (IIaA). Submetido à angioTC de aorta para programação do procedimento, evidenciando uma VAo trivalvular, fusão de seus folhetos e com calcificação importante (2743 Agatston), com extensão para a VSVE, causando redução da abertura valvar, e imagem de membrana subaórtica (MSAo). Sendo assim, o procedimento de TAVI foi contraindicado e paciente foi encaminhado para cirurgia corretiva da VAo. DISCUSSÃO: A EAo e EAoS são patologias que podem condicionar gradientes hemodinâmicos na VSVE. A EAoS é responsável por 1% de todas CC, sendo 15 a 20% de todos os defeitos fixos de obstrução à VSVE e até 65% tem associação com outras malformações, como a coarctação de aorta. Devido à presença da MSAo, esta pode estar aderida ao septo interventricular ou contornar VSVE, causando disfunção importante da valva aórtica (VAo). O ECOTT é o exame de escolha para diagnosticar EAoS, porém nos casos inconclusivos, a angioTC surge como um importante método de imagem adjuvante. A excelente resolução espacial da angioTC permite avaliação funcional e anatômica, sobretudo nos casos nos quais a membrana é muito fina e muito próxima do plano valvar, podendo assim dificultar a avaliação ao ECOTT. Estudo de grande coorte (Talwar e cols) associou miectomia septal com menos recorrências e com excelente sobrevida. Como tratamento paliativo, pode ser realizada valvoplastia percutânea com balão. (Figura 1) CONCLUSÃO: A estenose subaórtica permanece um diagnóstico raro e clinicamente desafiador na população adulta. Muitas vezes, uma combinação de modalidades de imagem é necessária para o diagnóstico diferencial de obstrução do fluxo na VSVE. A angioTC cardíaca é um método de alta resolução espacial, com papel relevante no diagnóstico das cardiopatias congênitas, impactando nas decisões terapêuticas.

The choice of palliative arterial switch operation as an alternative for selected cases in a single center: Experience and mid term results.

INTRODUCTION AND OBJECTIVE: There are various management options for newborns with single ventricle physiology, ventriculoarterial discordance and subaortic stenosis, classically involving the early pulmonary banding and aortic arch repair, the restricted bulboventriculer foramen enlargement or the Norwood and the Damus-Kaye-Stansel procedures. The aim of this study is to evaluate our preferred technique and comment on the midterm results of our clinical experience with palliative arterial switch operation (pASO) for a certain subset of patients. METHOD: We hereby retrospectively evaluate the charts of patients who went through pASO, as initial palliation through Fontan pathway, starting from 2014 till today. RESULTS: Ten patients underwent an initial palliative arterial switch procedure. Eight of 10 patients survived the operation and discharged. Seven of 10 patients completed Stage II and 1 patient reached the Fontan completion stage and the other six of ten (6/10) patients are doing well and waiting for the next stage of palliation. There are two mortalities in the series (2/10) and one patient lost to follow-up (1/10). CONCLUSION: In our opinion, the pASO can be considered as an alternative palliation option for patients with single ventricle physiology, transposition of the great arteries and systemic outflow tract obstruction despite longer cross clamp times compared to other methods, but It not only preserves systolic and diastolic ventricular function, but also provides a superior anatomic arrangement for following stages.

Palliative combined cutting and high-pressure balloon valvuloplasty in six dogs with severe, symptomatic subaortic stenosis.

INTRODUCTION/OBJECTIVES: Severe subaortic stenosis (SAS) is a congenital heart defect in dogs that often results in clinical signs and reduced survival. The objective of this study was to describe characteristics of dogs with severe, symptomatic SAS who underwent combined cutting and high-pressure balloon valvuloplasty (CB/HPBV). ANIMALS, MATERIALS, AND METHODS: Retrospective description of the clinical characteristics, CB/HPBV procedural deviations from reported methodology and outcomes in a series of six client-owned dogs with severe, symptomatic SAS. RESULTS: Breeds included two each of Newfoundland, Golden retriever, and German shepherd. Median age was 10.1 months (range: 5-72.3 months), and median weight was 25.5 kg (range: 21.8-36.4 kg). Before CB/HPBV, clinical signs were present in all dogs; four were managed for congestive heart failure (CHF). Three dogs had concurrent congenital heart disease. Median Doppler-estimated left ventricular outflow tract pressure gradient was pre-operatively 149.7 mmHg (range: 89.9-254.7 mmHg) and post-operatively 134.1 mmHg (range: 83.9-181.2 mmHg). Median aortoseptal angle was steep at 136° (range: 109-143°). Clinical improvement was documented in all dogs, based on temporary discontinuation of diuretics and/or owner-perceived reduction in clinical signs. At the time of writing, three dogs had died suddenly, one was euthanized because of recurrence of clinical signs, and one died in CHF. Median survival time was 26.4 months after procedure (range: 6.3-45.8 months). One dog remained alive at 44 months after procedure. CONCLUSIONS: Palliative CB/HPBV is a potential therapeutic option for dogs with severe, symptomatic SAS complicated by concurrent congenital heart disease, arrhythmias, or CHF.

Critical subaortic stenosis due to fibromuscular ridge in a 66-year-old woman: 2D-3D echocardiography findings.

Subvalvular aortic stenosis (SAS) can be either a fixed stenosis resulting from subaortic anatomical obstacle or a dynamic stenosis because of hypertrophic cardiomyopathy. Here, we report a patient with a rare subaortic circumferential fibromuscular tunnel who became unusually symptomatic in her sixth decade of life. Transthoracic and mostly transesophageal echocardiography with 3D application is the preferred diagnostic modality. Indications for surgery include symptoms and LVOT gradient of 50 mm Hg. Our purpose is an image focus on this infrequent case.

Unusual Ventriculo-Arterial Alignments and Connections.

In this issue of World Journal for Pediatric and Congenital Heart Surgery, Kari and colleagues present a case of anatomically corrected malposition {S, L, L} with ventricular septal defect and complex subaortic stenosis managed by staged biventricular repair. This rare case with interesting management strategy provides an opportunity to clarify this and related unusual ventriculo-arterial alignments.

Aortocaval compression resulting in sudden loss of consciousness and severe bradycardia and hypotension during cesarean section in a patient with subvalvular aortic stenosis.

BACKGROUND: Maternal cardiac arrest during cesarean section (CS) is an extremely rare but devastating complication. Preventing emergency events from developing into maternal cardiac arrest is one of the most challenging clinical scenarios. CASE PRESENTATION: A 35-year-old pregnant woman with subvalvular aortic stenosis who was scheduled for elective CS under epidural anesthesia, and experienced devastating supine hypotensive syndrome, but was successfully resuscitated after delivery. CONCLUSIONS: The performance of tilt position strictly or high-quality continue manual left uterine displacement (LUD) should be performed until the fetus is delivered, otherwise timely delivery of the fetus may be the best way to optimize the deadly condition.