Severe Left Ventricular Outflow Tract Research Articles

Transapical aortic valve replacement complicated by periaortic hematoma.

Abstract A 77-year-old woman with severe symptomatic aortic stenosis (aortic valve areaZ0.6 cm2 and mean gradientZ 53 mmHg) was deemed inoperable for surgical aortic valve replacement due to a porcelain aorta, and was scheduled for transcatheter aortic valve replacement via an apical approach. A 23 mm valve was selected based on a computerized axial tomographic angiography derived area of 360 mm2; valve oversizing was 15.4%. Baseline transesophageal echocardiography revealed severe protruding atheroma within the lumen of the ascending aorta. Immediately following deployment of the Sapien pericardial tissue valve (Edwards Life Sciences, Irvine, CA, USA), a large posterior periaortic hematoma was noted (1.3 cm in maximum width, and w4 cm in length) (see Fig. 1 and Videos 1, 2, 3, 4). Protamine was administered, and the hematoma was observed by transesophegeal echocardiography for w30 min with no change in size; function of the bioprosthetic valve was normal without regurgitation. In the immediate post-operative period, anti-platelet agents were withheld, and blood pressure was aggressively lowered. The remainder of her hospital course was uncomplicated, and a tranthoracic echocardiogram w1 month later demonstrated resolution of the hematoma. Periaortic hematoma and aortic root rupture are recognized potential major complications of transcatheter aortic valve replacement (1, 2). Severe left ventricular outflow tract calcification and annular over sizing are both risk factors for these complications. Careful transesophageal echocardiographic examination post valve deployment may lead to early recognition and treatment of this potentially fatal complication (3). In this case, presence of severe protruding atherosclerotic plaque pre-procedure likely predisposed to this complication.

Symptom assessment and exercise impairment in surgical decision making in hypertrophic obstructive cardiomyopathy: Relationship to outcomes

ObjectivesWe sought to assess the long-term outcomes in patients with hypertrophic cardiomyopathy and severe left ventricular outflow tract obstruction, in whom the decision regarding surgery (vs conservative management) was based on assessment of symptoms or exercise capacity. MethodsThis was an observational study of 1530 patients with hypertrophic cardiomyopathy (aged 50 ± 13 years, 63% were men) with severe left ventricular outflow tract obstruction (excluding those aged <18 years, with left ventricular ejection fraction <50%, and with left ventricular outflow tract gradient <30 mm Hg). A composite end point of death (excluding noncardiac causes) and/or implantable defibrillator discharge was assessed. ResultsCoronary artery disease, family history of hypertrophic cardiomyopathy, and syncope were present in 15%, 17%, and 18% of patients, respectively, whereas 73% patients were in New York Heart Association class II or greater. Mean left ventricular ejection fraction, basal septal thickness, and left ventricular outflow tract gradient (resting or provocable) were 62% ± 5%, 2.2 ± 1 cm, and 101 ± 39 mm Hg, respectively. A total of 858 patients (56%) underwent exercise echocardiography, of whom 503 (59%) had exercise capacity impairment. At 8.1 ± 6 years, 990 patients (65%) underwent surgical relief of left ventricular outflow tract obstruction, and 540 patients (35%) did not. There were 156 events (10%) (134 deaths), with 0% 30-day mortality in the surgical group. On multivariable Cox proportional hazard analysis, increasing age (hazard ratio [HR], 1.20), coronary artery disease (HR, 1.68), worse New York Heart Association class (HR, 1.46), and atrial fibrillation (HR, 1.90) predicted higher events, whereas surgery (time-dependent covariate HR, 0.57) was associated with improved event-free survival (all P < .01). ConclusionsIn patients with hypertrophic cardiomyopathy and severe left ventricular outflow tract obstruction, in whom the decision regarding surgery was based on the presence of intractable symptoms and impaired exercise capacity, surgery was associated with significant improvement in long-term composite outcomes.

Apical ballooning and cardiomyopathy in a melanoma patient treated with ipilimumab: a case of takotsubo-like syndrome.

Although animal studies have shown that the immunomodulator ipilimumab causes inflammation of the myocardium, clinically significant myocarditis has been observed only infrequently. We report a case of suspected acute coronary syndrome without a culprit lesion on cardiac angiography and takotsubo cardiomyopathy (TC)-like appearance on echocardiography in a patient with metastatic melanoma who received four standard doses of ipilimumab. Apical ballooning, hyperdynamic basal wall motion, systolic anterior motion of the mitral valve, and associated severe left ventricular outflow tract obstruction were present. Restaging with positron emission tomography-computed tomography done soon after discharge incidentally revealed increased fludeoxyglucose uptake in the apex. This case illustrates that a TC-like syndrome might be caused by autoimmune myocarditis after ipilimumab treatment although this was not biopsy-confirmed. Post-marketing surveillance should capture cardiac events occurring in patients treated with ipilimumab to better document and clarify a relationship to the drug, and biopsies should be considered. Physicians utilizing this novel agent should be aware of the potential for immune-related adverse events.

Myocardial T1 mapping in pediatric and congenital heart disease.

In addition to the depiction of anatomy and function, tissue characterization of the myocardium has emerged as an important asset of cardiac magnetic resonance (CMR). Among the tissue properties that are quantifiable by CMR is diffuse myocardial fibrosis. Diffuse fibrosis is regarded to be the common pathological pathway toward loss of myocardial function in many cardiac conditions, including congenital heart disease.1–4 As fibrosis seems to have a major role in myocardial failure and may be reversible,5–8 its assessment by CMR has the potential to transform the way we monitor and treat our patients.9 In this review, we describe the technical aspects of fibrosis quantification with CMR and outline past and potential applications in congenital and pediatric heart disease. Diffuse myocardial fibrosis, which is present to a varying degree in children with acquired and congenital heart disease, has been attributed to abnormal loading conditions, cyanosis, and genetic predisposition.1,7,10,11 Myocardial fibrosis manifests in the pressure-loaded left ventricle of infants and children with aortic stenosis and coarctation.1 In patients with tetralogy of Fallot right ventricular myofiber disorganization and interstitial fibrosis have been demonstrated histologically.2,12 Interestingly, right ventricular fibrosis not only occurs in late adult survivors, but is already present in infants with this condition.12 The changes in myocardial architecture observed with fibrotic remodeling can be detrimental to heart function: Fibrous endocardial thickening of the right ventricular infundibulum is a predictor of poor right ventricular function in patients after tetralogy of Fallot repair.3 In patients with tricuspid atresia myocardial fibrosis is associated with systolic ventricular dysfunction early in life.4,13 In addition to being a mediator of cardiac dysfunction, diffuse fibrosis is the substrate for electric instability and a risk factor for life-threatening ventricular arrhythmia.14 Antifibrotic drugs have …

Abstract 13729: Surgical Myectomy Results in Significantly Improved Long-term Event Rate in Hypertrophic Cardiomyopathy Patients With Severe Left Ventricular Outflow Tract Obstruction

Background: In hypertrophic cardiomyopathy (HCM) with severe left ventricular outflow tract obstruction (LVOTO), surgical myectomy (SM) performed for relief of intractable symptoms is safe and associated with excellent long-term symptom-free survival. In such patients, we sought to determine if SM also results in improvement of long-term outcomes. Methods: We studied 1530 HCM patients with severe LVOTO (50±13 years, 63% men) that were evaluated at our center [excluding &lt;18 years of age, LV ejection fraction (LVEF) &lt;50%, LVOT gradient &lt;30 mm Hg). Clinical, echocardiographic and surgical data were recorded. A composite endpoint of death and/or implantable defibrillator (ICD) discharge was recorded. Results: Hypertension, coronary artery disease (CAD), family history of HCM and syncope were present in 41%, 15%, 17% and 18%, while 73% patients were in Functional Class (FC) ≥ II. Mean LVEF, basal septal thickness, LVOT gradient (resting or provocable) and indexed left atrial dimensions were 62±5%, 2.2±1 cm, 101±39 mm Hg, 2.2±0.4 cm/m2. During 8±6 years of follow-up, 990 (65%) patients underwent SM (of which 65% were isolated SM), while 540 (35%) did not. 94 (6%) patients had alcohol septal ablation (66 in the non myectomy group), while 18% developed atrial fibrillation (AF), and 18% had ICD. There were 169 (11%) events (151 deaths), with 0% 30-day mortality in the SM group. On stepwise multivariable Cox Proportional Hazard analysis, increasing age (Hazard Ratio or HR 1.22 [1.06-1.40]), CAD (HR 1.57 [1.06-2.33]), worsening FC (HR 1.34 [1.05-1.71]) and AF (HR 1.73 [1.23-2.43]) predicted higher events, while SM as a time-dependent covariate (HR 0.58 [0.41-0.81]) was associated with improved event-free survival (all p&lt;0.01). Kaplan-Meier curve showing impact of SM on outcomes is shown in Figure. Conclusion: In HCM patients with severe LVOTO, SM is associated with significant improvement in long-term outcomes when compared to watchful waiting.

Abstract 351: Optimizing the Frequency of Surveillance Echocardiography in Clinically Stable Patients With Hypertrophic Cardiomyopathy

Background: Hypertrophic cardiomyopathy (HCM) affects 0.2% of the population and is an important cause of sudden cardiac death, heart failure and stroke. However, most patients with HCM experience normal longevity. Optimal longitudinal management of stable HCM patients is poorly defined, although consensus guidelines advise annual echocardiography (echo). We report the clinical impact of surveillance echo and a protocol to improve the efficiency of repeat testing in a cohort of HCM patients followed at one center. Methods and Results: A Standardized Clinical Assessment and Management Plan (SCAMP) was developed by clinicians, supported by a quality improvement (QI) team, to maximize the utility of cardiac imaging in patients with HCM. SCAMPs are an established approach to QI that aim to optimize outcomes and resource utilization through standardization of care. The HCM SCAMP was designed to achieve these goals through implementation of a practice algorithm, data capture at the time of outpatient encounters, and iterative analysis. This SCAMP recommended decreased frequency of surveillance echo in stable patients, from every 12 to every 24 months. Stability was defined as &gt;3 years since diagnosis, stable symptoms for ≥2 years, &gt;2 unchanged echos, and no family history of end stage HCM. All patients with HCM followed longitudinally in one HCM clinic from 7/17/13 to 12/15/13 (n=127) were enrolled. After excluding patients with a new diagnosis (n=16), no recent echo (n=15), or incomplete SCAMP documentation (n=1), 92 were included in this analysis. At enrollment, baseline echo (mean interval from prior echo: 15.8±5.8 months) identified clinically significant changes in 13 (14%) patients; including new pulmonary hypertension (n=6), and significant change (≥Δ50mmHg) or interval appearance of severe left ventricular outflow tract obstruction (LVOTO) (n=6). Clinical management was impacted by echo findings in 8/92 (9%); including medication adjustment (n=6), referral for exercise testing (n=2) and refined sudden cardiac death risk stratification (n=1). In the 79 patients with stable echo findings (86%), 25 (32%) were qualified by the SCAMP as clinically stable and were advised to undergo repeat echo in 24 (instead of 12) months. Providers adhered to this recommendation in 19 patients (76%). Reasons for deviating from recommendations and continuing annual echo in SCAMP defined stable patients included patient preference, atrial fibrillation, severe LVOTO, and pulmonary hypertension. Conclusions: In select clinically stable patients with HCM, repeat echo may be unlikely to identify significant changes or impact practice. In these patients, a decreased frequency of echocardiography could decrease resource utilization without compromising care. Ongoing data collection and better risk stratification tools may allow more targeted surveillance strategies in the future.

An unusual cause of left ventricular outflow tract obstruction: obstructive pannus on the left ventricular side of a mechanical mitral prosthesis.

A 37-year-old female, who had undergone mitral valve replacement with a monoleaflet mechanical prosthesis for mitral stenosis in 1997, was admitted to outpatient clinic with exertional dyspnea and chest discomfort over preceding 3 months. Her physical examination revealed mildly muffled prosthetic valve sounds, pansystolic murmur on lower left sternal border, and bilateral fine rales at the bases of the lungs. Her electrocardiography showed atrial fibrillation. Transthoracic echocardiography revealed severe left ventricular outflow tract (LVOT) obstruction with mean gradient of 42 mmHg (Fig. 1a, c; Video 1) and severe tricuspid regurgitation. Two-dimensional transesophageal echocardiography (2D-TEE) showed stenosis of mechanical mitral valve (MMV) with pressure half-time (PHT) of 157 ms (PHT was 98 ms in the previous echocardiography) and mean gradient of 13 mmHg. Aortic valve function and leaflet motion of MMV were normal, but there was a hyperechogenic appearance at the ventricular side of the MMV. With the transducer array at 120 , 2D-TEE revealed that the hyperechogenic mass on the ventricular side of the MMV caused narrowing of the LVOT (Fig. 2a; Video 2). Real-time three-dimensional transesophageal echocardiography (RT-3D-TEE) clearly delineated an eccentric semicircular pannus overgrowth which was located anteriorly on the ventricular side of the MMV (Fig. 2b; Video 3), resulting in LVOT obstruction (Fig. 2c; Video 4). Redo mitral valve replacement with a bileaflet mechanical prosthesis (no. 29, St. Jude Medical) and concomitant repair of the tricuspid valve were performed, and anteriorly eccentric semicircular pannus tissue was removed from the left ventricular side of the mitral prosthesis (Fig. 2d). Postoperative transthoracic echocardiography (TTE) revealed normal functioning mitral prosthetic valve with normal LVOT gradients (Fig. 1b, d; Video 5). Prosthetic heart valve obstruction caused by pannus formation is an uncommon but serious complication. Determination of the precise etiology of valvular obstruction is essential, as this can have an impact on the course of surgical versus thrombolytic therapy [1]. Although 2DTEE is the first method of choice in the diagnostic algorithm, exact visualization of pannus is almost impossible [2]. However, RT-3D-TEE is a very useful imaging technique and permits differentiation of pannus overgrowth from other MMV pathologies [3, 4]. Herein, we present an anteriorly localized eccentric pannus overgrowth on the left ventricular side of MMV, which is an uncommon cause of LVOT obstruction.

IMPLANTE DE CONDUCTO APICOAÓRTICO EN PACIENTE CON ESTENOSIS AÓRTICA SEVERA POST REEMPLAZO VALVULAR PROTÉSICO

Apicoaortic valved conduit implanted in a patient with severe aortic stenosis due to mismatched aortic valve prosthesis Introduction: The most frequent surgical procedure of severe left ventricular outflow tract obstruction due to severe aortic stenosis is aortic valve replacement, but there is an alternative and less conventional method described by Cooley used in patients with previous aortic valve replacement and severe aortic wall calcification that bypasses the left ventricular outflow tract through the implant of an apicoaortic valved con - duit. Clinical case: We describe the case of 42 years old patient with severe aortic stenosis due to mismatched valve prosthesis previously installed treated by of apicoaortic conduit implanted through left posterolateral thoracotomy.

FIRST RESULTS OF MICROSPHERE EMBOLIZATION AS AN ALTERNATIVE FOR ALCOHOL IN PERCUTANEOUS TRANSLUMINAL SEPTAL MYOCARDIAL ABLATION

Percutaneous transluminal septal myocardial ablation (PTSMA) using microsphere embolization instead of alcohol is a new interventional technique to treat patients with obstructive hypertrophic cardiomyopathy (HCM). Six HCM patients (age 68.4±6.3) with severe left ventricular outflow tract (LVOT)

A case of successful percutaneous transluminal septal myocardial ablation for heart failure due to severe left ventricular outflow obstruction with Mönckeberg’s arteriosclerosis, manifested after aortic valvular replacement

Although percutaneous transluminal septal myocardial ablation (PTSMA) has been the established treatment of symptomatic patients with hypertrophic obstructive cardiomyopathy (HOCM), the efficacy for specific HOCM is not elucidated. We report a successful case of PTSMA for heart failure with severe left ventricular outflow tract (LVOT) obstruction due to sigmoid-shaped interventricular septum and diffuse left ventricular hypertrophy with Mönckeberg's arteriosclerosis and aortic valvular stenosis. While the PTSMA relieved LVOT obstruction and symptoms in the acute phase, the modest recurrence was confirmed 6 months later, which is rare in the case of idiopathic HOCM. The possible mechanisms of LVOT obstruction and recurrence are discussed.

Hypertrophic Cardiomyopathy with Intermittent Free Mitral Regurgitation–A Surgical Dilemma

We describe the case of a 52-year-old woman presenting with non-ST elevation myocardial infarction, atrial fibrillation, and a new diagnosis of hypertrophic cardiomyopathy. Transesophageal echocardiography following hemodynamic deterioration revealed completely restricted mitral leaflet motion with free mitral regurgitation, and severe left ventricular outflow tract (LVOT) obstruction. Surgical intervention was considered; however, repeat imaging following a period of clinical stability revealed resolution of the findings suggesting a transient ischemic etiology. The case is supported by clinical and echocardiographic images with movie clips, and a discussion of the likely pathology in the context of the underlying condition.

Accessory Mitral Valve: A Rare Cause of Asymptomatic Severe Left Ventricular Outflow Tract Obstruction

Accessory mitral valve (AMV) is an extremely uncommon congenital cardiac anomaly and it is a rare cause of left ventricular outflow tract (LVOT) obstruction. Identification of asymptomatic patients with LVOT obstruction is uncommon since the symptoms usually become manifest when the gradient across the LVOT rises. We describe a rare case of asymptomatic AMV with severe LVOT obstruction in a 10-year old child with no other congenital or acquired cardiac defects who successful underwent surgery. This case emphasizes the importance of early surgical indication also in absence of symptoms when significant LVOT obstruction occurs.

Fabry's Disease with LVOT Obstruction: Diagnosis and Management

A 46-year-old male was diagnosed with hypertrophic cardiomyopathy (HCM) and severe left ventricular outflow tract (LVOT) obstruction. Genetic testing revealed that this was a "mimic of HCM" and the true diagnosis was Fabry's disease, although there were no other clinical features of Fabry's. Despite maximal medical therapy he remained symptomatic from the outflow tract gradient and required surgical myectomy for symptom relief. This case illustrates the diagnosis of an HCM mimic by genetic testing.

Circulation : Clinical Summaries

<i>Circulation</i> : Clinical Summaries

Simultaneous closure of the left atrial appendage and a severe LVOT: atrial shunt defect

A 72-year-old male with atrial fibrillation was transferred to our clinic for the evaluation of the interventional closure of the left atrial appendage (LAA) after a major bleeding complication under the novel anti-factor Xa inhibitor rivaroxaban. He had a history of biological aortic valve (AV) replacement and pericardial patch plastic of the left ventricular outflow tract (LVOT) after abscess forming infective endocarditis of the AV and LVOT ( Panel A , preoperative state, arrows …

Predictors of Long-Term Outcomes in Symptomatic Hypertrophic Obstructive Cardiomyopathy Patients Undergoing Surgical Relief of Left Ventricular Outflow Tract Obstruction

We report the predictors of long-term outcomes of symptomatic hypertrophic cardiomyopathy patients undergoing surgical relief of left ventricular outflow tract obstruction. We studied 699 consecutive patients who have hypertrophic cardiomyopathy with severe symptomatic left ventricular outflow tract obstruction (47±11 years, 63% male) intractable to maximal medical therapy, who were referred to a tertiary hospital between January 1997 and December 2007 for the surgical relief of left ventricular outflow tract obstruction. We excluded patients <18 years of age, those with an ejection fraction <50%, those with hypertensive heart disease of the elderly, and those with more than mild aortic or mitral stenosis. Clinical, echocardiographic, and Holter data were recorded. A composite end point of death, appropriate internal cardioverter defibrillator discharges, resuscitated from sudden death, documented stroke, and admission for congestive heart failure was recorded. During a mean follow-up of 6.2±3 years, 86 patients (12%) met the composite end point with 30-day, 1-year, and 2-year event rates of 0.7%, 2.8%, and 4.7%, respectively. The hard event rate (death, defibrillator discharge, and resuscitated from sudden death) at 30 days, 1 year, and 2 years was 0%, 1.5%, and 3%, respectively. Stepwise multivariable analysis identified residual postoperative atrial fibrillation (hazard ratio, 2.12; confidence interval, 1.37-3.34; P=0.001) and increasing age (hazard ratio, 1.49; confidence interval, 1.22-1.82; P=0.001) as independent predictors of long-term composite outcomes. Symptomatic adult hypertrophic cardiomyopathy patients undergoing surgery for the relief of left ventricular outflow tract obstruction have low event rates during long-term follow-up; worse outcomes are predicted by increasing age and the presence of residual atrial fibrillation during follow-up.

Aortic atresia: A case report

A 1 day-old male newborn with the diagnosis of pulmonary atresia revealed with fetal echocardiography was admitted because of tachypnea. He was acyanotic, and he had intercostal retractions. Physical examination revealed a systolic murmur (grade 2/6) best heard at the 4th left intercostal space. Peripheral pulses were palpable and oxygen saturation was 92%. No significant changes were detected on standard 12-lead-electrocardiogram. Postnatal transthoracic echocardiography revealed absence of left ventricular outflow with large perimembranous ventricular septal defect and normal size of right and left ventricles. There was retrograde filling of the ascending aorta via patent ductus arteriosus. Prostaglandin E1 infusion was initiated to maintain patent ductus arteriosus. Cardiac catheterization was performed to demonstrate aortic morphology. Aortography depicted a hypoplastic ascending aorta without any communication between aorta, and left ventricle. Coronary arteries had normal branching and originated from the atretic end of the ascending aorta. Hypoplastic ascending aorta seemed to be a main coronary artery trunk (Video-1, Figure 1). The patient was considered as unavailable for corrective surgery and died 3 days after his birth while under prostaglandin E1 infusion and appropriate supportive treatment. Most cases with aortic atresia with left ventricular hypoplasia are grouped in the category of hypoplastic left heart syndrome (1,2). Few cases with a normal left ventricular configuration have been published (3). Aortic atresia or severe aortic stenosis and left ventricular outflow tract obstruction with two functionally adequate ventricles are sometimes treated by Norwood palliation followed by late biventricular repair (4). Actually this anomaly is not compatible with life and most of the fetuses are expected to die in utero or during the neonatal period (3,4).

The role of Ross procedure in the management of congenital heart defects

The surgical strategy to manage multilevel left ventricular outflow tract diseases is changing constantly, however, the Ross-procedure has remained a standard method for 45 years. The aim of the study was to analyze early and mid-term results of Ross-procedure in congenital heart defects (single surgeon's results). From 2001 until 2011 a total of 63 patients (age, 28 days-21 years; mean: 10 years, weight 3.4-96 kg; mean, 8.8 kg) underwent Ross (n = 40), Ross-Konno (n = 17) or Ross-Konno-mitral (n = 6) procedures. Indication for Ross procedure was aortic regurgitation in 15 patients (associated with ventricular septum defect in 8 patients) and a predominant aortic stenosis in 25 patients. 17 patients with severe left ventricular outflow tract obstruction underwent Ross-Konno procedure. 6 patients with concomitant mitral valve disease (Shone syndrome, 3 patients; complete atrioventricular septal defect, 3 patients) were reconstructed by Ross-Konno-mitral valve procedure. Among Ross patients there were one early (cerebral complication) and one late death (homograft endocarditis) with a mean follow-up time of 7.4±1.8 years. Because of an early autograft endocarditis a 3-year-old boy underwent homograft implantation and was treated successfully with Bentall-procedure 9 years later. One patient with autograft regurgitation is waiting for reoperation. Among Ross-Konno patients there was no early or late death, and none of the patients underwent reoperation. In Ross-Konno-mitral patients there was one early death (28-day-old boy) and during a mean follow-up time of 2.5±1 years, and no reintervention or reoperation was needed in 5 patients. The results indicate a good outcome of Ross-, Ross-Konno-, Ross-Konno-mitral procedures in patients with congenital heart defects when surgery is performed by a highly experienced heart surgeon. In newborns, infants and small children Ross- and Ross-Konno procedures are the only methods for managing left ventricular outflow tract diseases. Concomitant severe mitral disease adds a high level of technical complexity to the Ross-Konno/mitral procedure, but it should be balanced against alternative strategies (eg. single ventricle palliation or transplantation).

Surgical removal of a left ventricular myxoma in an infant

Left ventricular (LV) myxoma is particularly rare in children and has not been reported in infants. A five-month-old baby presented with a myxoma arising from the anterior, lateral, and superior aspect of the LV, causing severe left ventricular outflow tract obstruction. The LV was accessed through the conal septum after opening the right ventricular outflow. The child had transient complete heart block in the postoperative period. There was no recurrence of tumor at the nine-month follow-up.

Perioperative management of an elderly patient of hypertrophic obstructive cardiomyopathy for knee arthroplasty and the role of peripheral nerve blocks

This case report exemplifies how the anaesthetic technique of general anesthesia with continuous bilateral femoral nerve block for bilateral knee arthroplasty was well chosen for the management of perioperative complications in an elderly patient with hypertrophic obstructive cardiomyopathy (HOCM). A 69-year-old female patient of HOCM was scheduled for bilateral total knee replacement. Echocardiography revealed severe left ventricular outflow tract obstruction with peak systolic gradient of 56 mmHg. The surgery was conducted under general anaesthesia with invasive monitoring and bilateral continuous femoral nerve blocks for postoperative analgesia. Postoperatively, she developed pulmonary oedema due to the liberal administration of fluids. This complication was successfully managed without interrupting the management of pain. Management of patients with HOCM for noncardiac surgery requires knowledge of variable presentation of two forms of disease. Also, this case report highlights the practical advantage of continuous femoral nerve block (CFNB)s over epidural anaesthesia.