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.

Severe Valvular Aortic Stenosis and Fixed Subvalvular Aortic Stenosis: A Rare and Challenging Combination.

A 58-year-old man with a history of hypertension presented with accelerating angina. Transthoracic echocardiography revealed a thickened aortic valve with pressure gradients and an estimated aortic valve area suggestive of mild aortic stenosis. Left heart catheterization demonstrated non-significant coronary artery disease. Pressure tracings showed a high left ventricular pressure and a mean gradient across the aortic valve of 69 mmHg. Subsequent transesophageal echocardiography revealed a subvalvular aortic stenosis that was secondary to the subaortic membrane, with severe valvular aortic stenosis. The patient underwent surgical resection of the subaortic membrane followed by bioprosthetic aortic valve replacement, with resolution of his symptoms. Video 1: Transesophageal echocardiography, five-chamber view, showing the calcified aortic valve and subaortic membrane. Video 2: Transesophageal echocardiography, long-axis view, showing aliasing of the aortic flow at valvular and subvalvular levels.

When Is It Better to Wait? Surgical Timing and Recurrence Risk for Children Undergoing Repair of Subaortic Stenosis.

Recurrence of subaortic stenosis (SAS) after surgery is common in children. The effects of patient characteristics and surgical timing on disease recurrence are largely unknown. We performed a retrospective study, assessing the relative effects of patient age and left ventricular outflow tract (LVOT) gradient on the need for reoperation for recurrent SAS. We included all children <20 years of age who underwent initial surgical resection of SAS at our center, January 2003-December 2013. Stratified logistic regression was performed, considering the effects of patient demographics, clinical characteristics, echocardiographic parameters, and operative technique, and clustering standard errors by surgeon. The multivariable model was used to simulate predicted probabilities of recurrent SAS for children at varying ages and baseline LVOT gradients. Sixty-three patients (38 males) underwent initial operation for SAS. Patients were followed for a median of 3.7 years (IQR 1.2-7.1). Twenty-one percent of patients (n = 13) underwent reoperation for SAS. Twelve were male. For every 10 mmHg increase in preoperative peak gradient in boys, the odds of reoperation for SAS doubled (OR 2.01, CI 1.5-2.72, p < 0.001), and for every additional 6 months of age, the odds of reoperation decreased by 14% (OR 0.86, CI 0.84-0.88, p < 0.001). Both younger age and higher preoperative outflow tract gradient are independently associated with risk of reoperation. Clinicians should consider the age and rate of LVOT gradient change-and not just the absolute gradient-in determining initial surgical timing.

Aortoseptal angle and pressure gradient reduction following balloon valvuloplasty in dogs with severe subaortic stenosis.

INTRODUCTION: To determine the relationship between aortoseptal angle (AoSA) and the short- and long-term systolic pressure gradient (PG) reduction following combined cutting and high-pressure balloon valvuloplasty (CB/HPBV) in dogs with severe subaortic stenosis. ANIMALS: Retrospective study of 22 client-owned dogs of various breeds with severe subaortic stenosis (mean left ventricular to aortic PG = 143 mmHg; range = 80-322 mmHg) that underwent CB/HPBV. MATERIALS AND METHODS: Initial angiographic and left apical and right-sided parasternal long-axis view echocardiographic video loops were used for measuring the angle between the plane of the interventricular septum and the longitudinal axis of the ascending aorta. The PG reduction ratio immediately after CB/HPBV and 6 and 12 months later were compared with AoSA. RESULTS: Weak correlations were observed for all instances of PG reduction ratio and AoSA type. Significantly greater mean differences of PG reduction ratio were observed for angles >160° than for angles <160° at 24 h (>160° mean: 54.45, standard error [SE]: ±3.8; <160° mean: 39.88, SE: ±2.09), 6 months (>160° mean: 57.73, SE: ±10.9; <160° mean: 28.22, SE: ±3.42), and 12 months (>160° mean: 76.11, SE: ±17.5; <160° mean: 27.61, SE: ±6.44; p=0.003). CONCLUSIONS: Dogs with AoSA >160° on right-sided parasternal long-axis view echocardiograms responded with a greater PG reduction following CB/HPBV than did dogs with AoSA <160°. This suggests that AoSA is associated with long-term outcomes of CB/HPBV, and measurement could help in the evaluation of dogs that are candidates for CB/HPBV.

Continuous spinal labor analgesia for two deliveries in a parturient with severe subvalvular aortic stenosis.

Various degrees of left ventricular outflow tract (LVOT) obstruction have been seen in patients with subvalvular aortic stenosis (SAS). Regional analgesia during labor for parturients with SAS is relatively contraindicated because it has a potential risk for hemodynamic instability due to sympathetic blockade as a result of vasodilation by local anesthetics. We thought continuous spinal analgesia (CSA) using an opioid and minimal doses of local anesthetic could provide more stable hemodynamic status. We demonstrate the management of a 28-year-old pregnant patient with SAS who received CSA for her two deliveries. For her first delivery (peak pressure gradient (∆P) between LV and aorta was approximately 55 mmHg), intrathecal fentanyl was used as a basal infusion, but we needed a small amount of bupivacaine to provide supplemental intrathecal analgesia as labor progressed. Although there were mild fluctuations in hemodynamics, she was asymptomatic. For her second delivery (∆P between LV and aorta was approximately 90 mmHg), minimal doses of continuous bupivacaine were used as a basal infusion. For her additional analgesic requests, bolus co-administration of fentanyl was effective. There were no fluctuations in her hemodynamics. Although her SAS in her second pregnancy was more severe than in the first, her hemodynamics exhibited less fluctuation during the second delivery with this method. In conclusion, CSA using fentanyl combined with minimal doses of bupivacaine provided satisfactory analgesia and stable hemodynamics in parturient with severe SAS.

A modified technique of Konno aortoventriculoplasty for redo aortic valve replacement procedures.

This article puts forward a modified technique of Konno aortoventriculoplasty for repeat procedures. After incision of the ventricular septum, this approach involves aortic valve replacement using a mechanical valved conduit, reimplantation of the coronary arteries, and graft replacement of the ascending aorta. This modification allows the removal of the diseased ascending aortic wall caused by a previous patch enlargement or poststenotic dilation.

Congenital discrete subaortic stenosis in pregnancy: case report and literature review.

Today's growing numbers of pregnant patients with congenital heart defects presents challenges for cardiologists treating adult patients. The case is presented of a 19-year-old woman who was admitted with a threatened miscarriage in the third trimester and was diagnosed with an incomplete atrioventricular canal, cleft mitral valve with severe regurgitation, and severe congenital subaortic stenosis caused by a subaortic membrane. She was managed conservatively, delivered via cesarean section, and then underwent surgical repair of all defects with good outcome. Here, a review is provided of the literature on the natural course, complications, and treatment strategies of subaortic stenosis, together with a summary of recommendations for the management of the condition.

Miomectomía septal en membrana subaórtica recurrente / Septal myectomy in recurrent subaortic membrane

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Surgical management of subaortic stenosis.

A 63-year-old male patient with subaortic stenosis (Pmax 105 mmHg, Pmean 55 mmHg) and an aneurysm of the ascending aorta was referred to our hospital due to progressive angina pectoris. Transesophageal echocardiography demonstrated high and turbulent subaortic flow velocities. A calcified subaortic membrane was identified. The membrane was removed and the aneurysm was treated with a Bentall procedure. The patient recovered smoothly from surgery and was doing well 6 months after discharge.

The natural history of congenital subaortic stenosis.

BACKGROUND: Congenital subaortic stenosis (SAS) has a high recurrence rate after surgical correction. Aortic valve disease frequently coexists with SAS, being its occurrence and progression unpredictable. We aimed to assess predictors of SAS recurrence and aortic valve disease occurrence and progression. METHODS: Retrospective analysis of 51 patients with SAS followed in a congenital heart disease clinic of a tertiary care hospital from July 1982 to October 2009. RESULTS: The mean age at SAS diagnosis was 15 years and 28 patients (54.9%) were men. Aortic regurgitation was present in 23 (45.1%) patients. Thirty-four (66.7%) patients were submitted to SAS surgical correction, six (17.6%) of them also with aortic valve repair/replacement. Eighteen (52.9%) patients had SAS recurrence and eight patients (23.5%) underwent reoperation. A shorter time from diagnosis to surgery (odds ratio [OR] 0.98 [95% confidence interval, CI 0.96-1.00]; P= 0.04) and a left ventricular outflow tract (LVOT) peak pressure gradient at diagnosis > 50 mm Hg (OR 2.63 [95% CI 1.52-4.53]; P= 0.05) were independent predictors of SAS recurrence. At the end of follow-up, 37 patients (72.5%) had some form of aortic valve disease and 24 patients (47.1%) had progression for or of the aortic valve disease. Higher LVOT peak pressure gradient at diagnosis (OR 1.10 [95% CI 1.01-1.21]; P= 0.05) and higher left ventricular mass (OR 1.04 [95% CI 1.01-1.07]; P= 0.014) were independent predictors of aortic valve disease. Predictors of aortic valve disease progression were not found. CONCLUSIONS: SAS recurrence occurred mainly in patients operated sooner and with a high LVOT peak pressure gradients, suggesting a more severe form of disease. A more turbulent systolic jet at LVOT was probably one of the main causes of aortic valve damage. Aortic valve disease progression occurred independently of surgery and SAS recurrence. All LVOT morphological anomalies should be detected and corrected in order to prevent SAS recurrence and aortic valve disease.

The dilemma of subaortic stenosis--a single center experience of 15 years with a review of the literature.

OBJECTIVE: The term "subaortic stenosis" includes a variety of obstructions of the left ventricular outflow tract (LVOT), ranging from a short (discrete) subvalvular membrane to long, tunnel-like narrowing. An association with other congenital lesions is frequent. We reviewed the reported literature and describe our results, analyzing the nomenclature of and risk factors for restenosis after surgical treatment. METHODS: From 1994 to 2009, 81 children (53 males, 28 females; median age: 57 months, range [ R]: 5-204) underwent surgical relief of a subaortic stenosis. Patients were divided, according to pathology, into short segment (group A, n = 42) and complex obstructions (group B, n = 39), with the latter including long segment stenosis and/or associated anomalies such as aortic coarctation, interrupted aortic arch or Shone's complex. RESULTS: Surgery resulted in a significant reduction of the gradient between the left ventricle and the aorta in both groups (Δ P group A: 51 ± 28 mmHg, group B: 46 ± 25 mmHg). There was no operative mortality. One patient died in the early postoperative period due to pericardial tamponade. Median follow-up was 90 months (R = 0.5-187). Twenty-five (31%) patients required reoperation because of recurrent stenosis after a median of 43 months (R = 0.5-128). Seven (16%) patients belonging to group A developed restenosis, and 18 (46%) in group B. Freedom from reoperation for all patients was 60% after 10 years. 10 (40%) of the patients of group B were ultimately treated with a Ross-Konno reconstruction of the LVOT. CONCLUSION: Despite adequate surgical resection, recurrence of subaortic stenosis within several years after initial surgical treatment is frequent, especially in patients with complex lesions. In cases requiring reoperation, the surgical therapy is often extensive, and even includes Ross-Konno reconstruction of the LVOT.

Correlates and causes of death in patients with severe symptomatic aortic stenosis who are not eligible to participate in a clinical trial of transcatheter aortic valve implantation.

BACKGROUND: Transcatheter aortic valve implantation is currently being evaluated in patients with severe aortic stenosis who are considered high-risk surgical candidates. This study aimed to detect incidences, causes, and correlates of mortality in patients ineligible to participate in transcatheter aortic valve implantation studies. METHODS AND RESULTS: From April 2007 to July 2009, a cohort of 362 patients with severe aortic stenosis were screened and did not meet the inclusion/exclusion criteria necessary to participate in a transcatheter aortic valve implantation trial. These patients were classified into 2 groups: group 1 (medical): 274 (75.7%): 97 (35.4%) treated medically and 177 (64.6%) treated with balloon aortic valvuloplasty; and group 2 (surgical): 88 (24.3%). The medical/balloon aortic valvuloplasty group had significantly higher clinical risk compared with the surgical group, with significantly higher Society of Thoracic Surgeons score (12.8±7.0 versus 8.5±5.1; P<0.001) and logistic European System for Cardiac Operative Risk Evaluation (EuroSCORE) (42.4±22.8 versus 24.4±18.1; P<0.001). The medical/balloon aortic valvuloplasty group had a higher New York Heart Association functional class, incidence of renal failure, and lower ejection fraction. During median follow-up of 377.5 days, mortality in the medical/balloon aortic valvuloplasty group was 102 (37.2%), and during median follow-up of 386 days, mortality in the surgical group was 19 (21.5%). Multivariable adjustment analysis identified renal failure (hazard ratio [HR]: 5.60), New York Heart Association class IV (HR: 5.88), and aortic systolic pressure (HR: 0.99) as independent correlates for mortality in the medical group, whereas renal failure (HR: 7.45), Society of Thoracic Surgeons score (STS; HR: 1.09) and logistic EuroSCORE (HR: 1.45) were correlates of mortality in the in the surgical group. CONCLUSIONS: Patients with severe symptomatic aortic stenosis not included in transcatheter aortic valve implantation trials do poorly and have extremely high mortality rates, especially in nonsurgical groups, and loss of quality of life in surgical groups.

Restrictive ventricular septal defect and critical subaortic stenosis in tetralogy of Fallot.

INTRODUCTION: Tetralogy of Fallot is characterized by a non-restrictive malalignment ventricular septal defect (VSD), an overriding aorta and right ventricular outflow tract obstruction resulting from anterior deviation of the infundibular septum. Due to the large VSD, systolic pressures are equal in both ventricles. In rare cases, redundant tricuspid valve tissue may prolapse into the VSD resulting in restriction of the defect size and causing suprasystemic pressure in the right ventricle. Subaortic obstruction by prolapse of the redundant tricuspid tissue into the left ventricular outflow tract, although theoretically possible in this situation, has not been described yet in the literature. CASE REPORT: We report on a newborn with tetralogy of Fallot presenting with cyanosis and severe arterial hypotension a few hours after birth. Echocardiography revealed redundant hammock-like accessory tricuspid valve tissue almost completely occluding the originally large VSD. Suprasystemic pressure in the right ventricle resulted in protrusion of tricuspid valve tissue across the VSD and thereby caused severe left ventricular outflow tract obstruction. Emergency pulmonary balloon valvuloplasty performed for decompression of the right ventricle, reduced prolapse of tricuspid tissue into the left ventricular outflow tract and resulted in significant improvement of pulmonary and systemic blood flow. CONCLUSION: In tetralogy of Fallot, accessory tricuspid valve tissue may obstruct the VSD as well as the left ventricular outflow tract resulting in a life threatening condition in newborns shortly after birth.

Treatment of severe valvular aortic stenosis and subvalvular discrete subaortic stenosis and septal hypertrophy with Percutaneous CoreValve Aortic Valve Implantation.

BACKGROUND: Percutaneous aortic valve implantation (PAVI) is a procedure gaining popularity and becoming more widely used for the treatment of patients with severe aortic stenosis who are at high risk for surgery. Here we show, for the first time, that a successful and complete elimination of both valvular and subvalvular pressure gradients can be achieved with a slight modification of the valve implantation technique. METHODS AND RESULTS: A 91-year-old woman presented with shortness of breath at rest, effort angina, and pulmonary congestion. Echocardiography revealed calcified aortic stenosis with a peak gradient of 75 mm Hg across the valve, and discrete subaortic stenosis (DSS) and marked hypertrophy of the basal septum with systolic anterior motion of the mitral valve (SAM). The intra ventricular gradient had a dynamic pattern across the DSS and the septal hypertrophy and measured 75 mm Hg. The total gradient across the left ventricular outflow (valvular and subvalvular) was 125 mmHg. PAVI with a 23 mm CoreValve was performed with an intentional lower positioning of the valve towards the LV outflow tract; so that the valve struts cover the subaortic membrane and part of the thickened basal septum. At the end of the procedure, the SAM disappeared, and the left ventricular ouflow was widely open. At 1 month follow up the patient was asymptomatic, no pressure gradient was measured between the LV apex and the aorta. CONCLUSIONS: This is the first report of successful treatment of severe valvular aortic stenosis and combined subvalvular aortic stenosis due to DSS and septal hypertrophy with SAM with percutaneous aortic valve implantation.

Aorto-septal angle in Boxer dogs with subaortic stenosis: an echocardiographic study.

The present study was designed to determine the aorto-septal angle (AoSA) in Boxer dogs with or without subaortic stenosis (SAS) by using two-dimensional echocardiography. Forty-five Boxer dogs were prospectively included in the study. The AoSA was steeper in the group with SAS than in healthy Boxers with a mean difference of 10 degrees . According to the proposed regression model, the AoSA is associated with SAS in Boxers, particularly because it becomes steeper as SAS severity increases. Several studies in humans demonstrate that small changes in the AoSA produce important changes in septal shear stress, which in turn causes proliferation of the endocardial cells resulting in subaortic obstruction. A definite conclusion about the role of the AoSA on the formation and/or progression of subvalvular lesions in Boxers cannot be drawn from the data analysed due to the transversal nature of the observations.

Predictors of survival after aortic valve replacement in patients with low-flow and high-gradient aortic stenosis.

AIMS: To identify predictors of survival following aortic valve replacement (AVR) in patients with low-flow and high-gradient aortic stenosis (AS). METHODS AND RESULTS: Eighty-six patients (aged 71 +/- 10 years) with severe AS [aortic valve mean pressure gradient >40 mmHg or valve area <1.0 cm(2)] and left ventricular (LV) dysfunction [ejection fraction (EF) <50%] underwent AVR. Cox proportional hazards were used to identify independent clinical and echocardiographic predictors of mortality. Operative (30-day) mortality was 10%. Peri-operative mortality was associated with lower mean LVEF, higher mitral E:A ratio, peak systolic pulmonary artery pressure (PSPAP), and serum creatinine (by 12%, 2.3, 28 mmHg, and 74 mmol/L, respectively, all P < 0.001), NYHA class III-IV (100 vs. 65%), concomitant CABG (89 vs. 55%), urgent surgery (78 vs. 35%), and longer bypass-time (by 28 min, all P < 0.05). Mortality at 4 years was 17%. Univariate predictors [hazard ratio (HR)] of 4-year mortality were: lower EF (HR 0.68 per % increase, P < 0.001), presence of restrictive LV filling (HR: 3.52, P < 0.001), raised PSPAP (HR: 1.07, P < 0.001), and CABG (HR: 4.93, P = 0.037). However, only low EF (<40%, HR 0.74, P = 0.030), the presence of restrictive filling (HR 1.77, P = 0.033), and raised PSPAP (>45 mmHg, HR 2.71, P = 0.010) remained as independent predictors after multivariate analysis. CONCLUSION: The severity of pre-operative systolic and diastolic LV dysfunction is the major predictor of mortality following AVR for low-flow and high-gradient AS.