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1.
JACC Case Rep ; 29(18): 102555, 2024 Sep 18.
Article in English | MEDLINE | ID: mdl-39359989

ABSTRACT

A coronary cameral fistula (CCF) is an abnormal communication between a coronary artery and any of the 4 cardiac chambers. Although congenital cases are more common, acquired CCFs, particularly after cardiac surgery, are rare. We present an unusual case of acquired CCF that occurred after bioprosthetic aortic valve replacement and septal myectomy and that mimics prosthetic regurgitation.

2.
J Am Heart Assoc ; : e036565, 2024 Oct 08.
Article in English | MEDLINE | ID: mdl-39377214

ABSTRACT

BACKGROUND: The clinical characteristics and survival outcomes of patients who underwent concomitant coronary artery bypass grafting during septal myectomy have not been well studied. METHODS AND RESULTS: We reviewed patients who underwent both septal myectomy and coronary artery bypass grafting from 2009 to 2020. Causes of concomitant grafting and their impact on survival were analyzed. The median follow-up period was 5.1 years. A total of 320 patients underwent both grafting and myectomy. Of these, 69.7% and 28.1% underwent grafting attributed to atherosclerotic coronary artery disease and myocardial bridging, respectively. Patients who underwent grafting for coronary artery disease tended to be older, had a longer bypass time, and required more grafts compared with patients undergoing procedures because of myocardial bridging (all P<0.05). Postoperatively, the left ventricular outflow gradient significantly decreased from 85.4 mm Hg to 12.8 mm Hg (P<0.001) without perioperative death. The cumulative survival rates were 96.2% and 97.6% at 5 years in the coronary artery disease and myocardial bridging groups, respectively, and they were comparable to that of general myectomy cohort (hazard ratio [HR], 1.06 [95% CI, 0.47-2.36], P=0.895 and HR 0.75 [95% CI, 0.23-2.46], P=0.636, respectively). Sudden death accounted for 45.5% (5 of 11) of postoperative mortality. Analysis of composite end point events showed decreased morbidity with at least one arterial graft in the overall cohort (HR, 0.47 [95% CI, 0.23-0.94], P=0.034). CONCLUSIONS: Concomitant grafting in septal myectomy was found to be a safe procedure. Patients who underwent such surgery experienced favorable postoperative outcomes comparable to those who underwent septal myectomy alone, with a 5-year survival rate of >95% and improved functional class of >90%.

3.
Article in English | MEDLINE | ID: mdl-39389416

ABSTRACT

BACKGROUND: Surgical septal reduction is sometimes avoided in older adults due to anticipated high operative risk. OBJECTIVE: To compare the clinical and echocardiographic characteristics of young and older patients undergoing septal myectomy for oHCM and assess differences in early and late postoperative outcomes. METHODS: 2663 patients with oHCM underwent transaortic septal myectomy between 2000 and 2021 and were categorized by age: 18-64, 65-74, and ≥75 years. RESULTS: Median age at the time of surgery increased over the study interval. Female sex (p<0.001), hypertension p<0.001), and diabetes (p=0.004) were more prevalent in older patients, but extent of functional limitation (NYHA) was similar (p=0.092). Elderly patients had thinner septal and posterior walls (p<0.001, p=0.006) and less prominent asymmetry (p<0.001). They are less likely to have positive genetic testing. Hospital mortality was 0.2%, 0.5%, and 1.3% in patients <65, 65-74, and ≥75 (p=0.06), and five-year survival rates were 97%, 93%, and 91%. Septal-to-posterior wall thickness ratio significantly correlated with increased mortality in patients >65, but not in patients <65 (p=0.92). Most of the patients reported improved quality of life following myectomy. CONCLUSION: Clinical characteristics of oHCM in older patients differ from those in younger ones. More symmetric but less extensive ventricular hypertrophy and less positive genetic testing suggests that HCM has distinct clinical and morphological variants in the elderly. Septal myectomy is safe in older patients, but the presence of LV wall asymmetry portends a poorer prognosis.

4.
J Cardiovasc Dev Dis ; 11(9)2024 Sep 18.
Article in English | MEDLINE | ID: mdl-39330348

ABSTRACT

Hypertrophic cardiomyopathy (HCM) is characterized by excessive growth of myocardial tissue, most commonly due to genetic mutations in sarcomere proteins. This can lead to complications such as heart failure, mitral regurgitation, syncope, arrhythmias, sudden cardiac death, and myocardial ischemia. While we have come a long way in our understanding of the pathophysiology, genetics, and epidemiology of HCM, the past 10 years have seen significant advancements in diagnosis and treatment. As the body of evidence on hypertrophic cardiomyopathy continues to grow, a comprehensive review of the current literature is an invaluable resource in organizing this knowledge. By doing so, the vast progress that has been made thus far will be widely available to all experts in the field. This review provides a comprehensive analysis of the scientific literature, exploring both well-established and cutting-edge diagnostic and therapeutic options. It also presents a unique perspective by incorporating topics such as exercise testing, genetic testing, radiofrequency ablation, risk stratification, and symptomatic management in non-obstructive HCM. Lastly, this review highlights areas where current and future research is at the forefront of innovation in hypertrophic cardiomyopathy.

5.
J Cardiovasc Dev Dis ; 11(9)2024 Sep 20.
Article in English | MEDLINE | ID: mdl-39330351

ABSTRACT

Anderson-Fabry disease (AFD) results from decreased enzyme activity of lysosomal enzymes and intralysosomal storage of nonhydrolyzed forms. Cardiovascular complications, mainly in the form of HCM, contribute substantially to AFD patient mortality. Here, we report three new cases of obstructive HCM (HOCM) in nonclassical presentations of AFD and isolated cardiac involvement. In all three cases, the diagnosis of AFD was made postoperatively by routine genetic and morphological testing. Together with previously published cases, this report illustrates the potential safety and beneficial effect of septal surgical myectomy in patients with AFD-HOCM, as well as underlines the need for more thorough screening for clinical signs of AFD-associated cardiomyopathy and GLA variants among patients with HOCM.

6.
JTCVS Open ; 20: 29-36, 2024 Aug.
Article in English | MEDLINE | ID: mdl-39296451

ABSTRACT

Background: Patients with hypertrophic cardiomyopathy (HCM) are at increased risk of developing cardiac arrhythmias and have a high prevalence of cardiac implantable electronic device (CIED) use. Tricuspid regurgitation (TR) is a potential complication of device leads and can be severe enough to prompt surgical intervention. Methods: We identified 21 consecutive patients who underwent tricuspid valve (TV) surgery for device lead-induced TR late following septal myectomy (SM) for obstructive HCM. The primary endpoint was long-term all-cause mortality. Results: The median patient age was 63 years (range, 55-71 years), 19 patients (91%) had New York Heart Association class III or IV limitation, and all patients were receiving diuretics for right heart failure. The median interval between device implantation and TV surgery was 4 years (range, 1.5-8.5 years). Eight patients (38%) underwent pacemaker implantation due to complete heart block following SM. Preoperatively, TR was severe in 81% of the patients. The primary mechanism of lead-induced TR was leaflet impingement without adherence (n = 15; 75%). Nine patients (43%) underwent TV replacement, and 12 patients (57%) underwent repair. Only 1 patient died early postoperatively. Patients with lead-induced TR had markedly reduced long-term survival compared to the overall population of patients undergoing SM; 5-year survival was 58%, compared to 96% for the contemporary SM group. Conclusions: Late lead-induced TR is a potential complication of CIEDs in patients with HCM who have undergone SM. Although TV repair and replacement can be done with acceptable early mortality, late patient survival is poor.

7.
Heliyon ; 10(18): e36613, 2024 Sep 30.
Article in English | MEDLINE | ID: mdl-39309828

ABSTRACT

Background: Unilateral superior oblique palsy (SOP) is the most common isolated cranial nerve palsy. This report looks at the results and safety of inferior oblique (IO) myectomy for SOP. Methods: A retrospective chart review of patients with fusional ability who underwent IO myectomy for unilateral SOP over a 15-year period, at one of our university-associated health care centers. Primary outcomes were: distance hypertropia and excyclotropia in primary position, inferior oblique overaction (IOOA) and the correlation between the age at surgery and the residual distance hypertropia. Results: A total of 73 patients with unilateral SOP who underwent IO myectomy were included in the analysis. Mean pre-operative values were: distance hypertropia: 15.41 ± 6.8 Prism Diopters (range 4-30 PD), IOOA: +2.2 ± 0.7 (range 0-3.5) and excyclotropia: +5.34 ± 3.6° (range 0-12°). Mean post-operative values were: distance hypertropia: 3.7 ± 3.4PD [0-14PD], IOOA: -0.1 ± 0.8 [(-2)-2] and excyclotropia: +1.1 ± 2.3°[(-5)-10]. The younger age group (0-20 years old) had a trend toward smaller preoperative distance hypertropia (P = 0.051), and a significantly smaller distance hypertropia post-operatively (p = 0.007). There was no case of ocular complication. Conclusions: On average, IO myectomy results in a 11.7PD reduction of the distance hypertropia, and 4° reduction of excyclotropia, with an effective reduction of IOOA. While these results are comparable to other surgical methods reported in the literature, they underline an efficacy without the risks of complications such as anti-elevation syndrome and possible scleral perforation. The younger age group had a trend toward smaller preoperative deviation, and better outcomes compared to the older age group.

8.
J Mol Cell Cardiol ; 196: 26-34, 2024 Sep 08.
Article in English | MEDLINE | ID: mdl-39255898

ABSTRACT

BACKGROUND: Hypertrophic cardiomyopathy (HCM) is a common genetic heart disease. Women with HCM tend to have a later onset but more severe disease course. However, the underlying pathobiological mechanisms for these differences remain unknown. METHODS: Myectomy samples from 97 patients (53 males/44 females) with symptomatic obstructive HCM and 23 control cardiac tissues were included in this study. RNA-sequencing was performed on all samples. Mass spectrometry-based proteomics and phosphoproteomics was performed on a representative subset of samples. RESULTS: The transcriptome, proteome, and phosphoproteome was similar between sexes and did not separate on PCA plotting. Overall, there were 482 differentially expressed genes (DEGs) between control females and control males while there were only 53 DEGs between HCM females and HCM males. There were 1983 DEGs between HCM females and control females compared to 1064 DEGs between HCM males and control males. Additionally, there was increased transcriptional downregulation of hypertrophy pathways in HCM females and in HCM males. HCM females had 119 differentially expressed proteins compared to control females while HCM males only had 27 compared to control males. Finally, the phosphoproteome showed females had 341 differentially phosphorylated proteins (DPPs) compared to controls while males only had 184. Interestingly, there was hypophosphorylation and inactivation of hypertrophy pathways in females but hyperphosphorylation and activation in males. CONCLUSION: There are subtle, but biologically relevant differences in the multi-omics profile of HCM. This study provides the most comprehensive atlas of sex-specific differences in the transcriptome, proteome, and phosphoproteome present at the time of surgical myectomy for obstructive HCM.

9.
Article in English | MEDLINE | ID: mdl-39226213

ABSTRACT

OBJECTIVES: To assess changes in the results of septal myectomy (SM) following introduction of three-dimensional (3D) imaging and 3D printing in surgical interventions planning and performing in the single-centre settings. METHODS: Between January 2007 and March 2022, 268 consecutive symptomatic patients with hypertrophic obstructive cardiomyopathy and peak pressure gradient at obstruction area ≥50 mmHg underwent conventional SM (n = 112) or SM with heart 3D modelling (n = 156). RESULTS: For comparative analysis, we used propensity score matching (PSM) by 14 variables and there were formed group 1PSM (conventional SM, n = 77) and group 2PSM (3D-modelled SM, n = 77). It was noted for group 2PSM: larger mean resected myocardium mass [10.0 (standard deviation 4.3) vs 5.2 (standard deviation 2.7) g], P < 0.001, no mitral valve replacement cases [0 vs 28 (36.4%), P < 0.001], no iatrogenic ventricular septal defects cases [0 vs 6 (7.8%), P = 0.028], lower rate of major complications [6 (7.8%) vs 17 (22.1%), P = 0.011], smaller residual peak systolic gradient at the obstruction level [7.0 (5.0-9.0) vs 11.0 (7.0-16.0) mmHg, P < 0.001]. During the long-term follow-up, it was noted for group 2PSM as compared to group 1PSM: lower 5-year cumulative incidence of major adverse cardiovascular events [3.8% (95% confidence interval 0.7-11.7%) vs 16.9% (9.5-26.1%), P = 0.007] and cardiac-related death [3.8% (95% confidence interval 0.7-11.7%) vs 13% (95% confidence interval 6.6-21.6%), P = 0.05]. CONCLUSIONS: SM based on 3D virtual and printed heart models is more effective than conventional SM.

10.
Cureus ; 16(6): e61976, 2024 Jun.
Article in English | MEDLINE | ID: mdl-39247483

ABSTRACT

Systemic lupus erythematosus (SLE) is an autoimmune disease that can affect multiple organ systems. Among these, the heart, including the pericardium, conduction system, myocardium, valves, and coronary arteries, can be affected. Hypertrophic cardiomyopathy (HCM) is a myocardial disease caused mainly by genetic mutation. The association between SLE and HCM is still unclear. We are reporting a case of a 25-year-old female with SLE with end-stage renal disease (ESRD) due to lupus nephritis, who was found to have hypertrophic obstructive cardiomyopathy (HOCM) on the echocardiogram and required septal myectomy. She presented to the hospital with dyspnea and was admitted as a hypertensive emergency with pulmonary edema, which required intubation and admission to the cardiac intensive care unit (CICU). She underwent urgent hemodialysis and blood pressure medication adjustment and then improved and was discharged home. Based on the literature review, 10 cases of SLE and HCM were reported, and the underlying mechanisms linking SLE and HCM remain unclear. Further studies are warranted for a better understanding of the association between SLE and HCM.

11.
Cureus ; 16(8): e66827, 2024 Aug.
Article in English | MEDLINE | ID: mdl-39280574

ABSTRACT

Hypertrophic cardiomyopathy encompasses a broad spectrum of muscular diseases that involve not only the interventricular septum and the left ventricular outflow tract but also the papillary muscles and the mitral valve apparatus. This article presents the successful surgical treatment of two patients with generalized hypertrophic cardiomyopathy with hypertrophy of the papillary muscles without severe septal hypertrophy: one with a history of unsuccessful chemical ablation and coronary disease following an interventional event, and another young patient without a history of chronic degenerative diseases who developed hypertrophic cardiomyopathy during her third pregnancy. Both patients with left ventricular outflow tract gradients greater than 55 mmHg and those who underwent surgical treatment had a gradient of less than 10 mmHg.

12.
Am J Cardiol ; 227: 48-56, 2024 Sep 15.
Article in English | MEDLINE | ID: mdl-39094946

ABSTRACT

Transesophageal echocardiography (TEE) plays an important role for real-time procedural guidance during surgical smyectomy (SM) for hypertrophic obstructive cardiomyopathy (HOCM). We aimed to compare (1) interventricular septum (IVS) thickness using 2- (2D) and 3-dimensional (3D) intraoperative TEE and preoperative cardiac magnetic resonance (CMR) and (2) mitral valve (MV) leaflet length using 2D, 3D TEE, automatic quantification of mitral valve (AMVQ) and preoperative CMR. We prospectively studied 50 patients with HOCM (age 59 ± 12 years, 44% men) who underwent SM during 2018 to 2019. The maximal basal, mid, and distal anteroseptum (AS) and inferoseptum (IS) were measured by multiplanar 3D reconstruction on TEE and by short-axis imaging on preoperative CMR and classified as mild (≤18 mm), moderate (18 to 25 mm), or severe (≥25 mm) groups based on AS and IS thickness on CMR. MV leaflet lengths were evaluated by preoperative CMR and intraprocedural 2D TEE, zoom 3D TEE, and AMVQ (EchoPAC, General Electric, Wisconsin). There was a moderate correlation between AS and IS thickness on 3D TEE and CMR (R2 = 0.46, p <0.01 and R2 = 0.41, p <0.01, respectively), with 3D TEE showing an average overestimation of 3.8 and 4.7 mm versus CMR. The 3D TEE overestimated 14 patients (56%) with mild thickness as moderate and 5 patients (22%) with moderate thickness as severe. Assuming 3D TEE as the gold standard, the closest correlation for anterior mitral leaflet length was with CMR (average overestimation by CMR of 0.5 mm [root mean square deviation (RMSE%) 17]), intermediate correlation with 2D TEE (average deviation of 0.6 mm [RMSE% 21]) and no correlation with AMVQ (average deviation of 0.7 mm [RMSE% 24]). In conclusion, 3D TEE overestimates IVS thickness versus CMR in patients with HOCM who underwent SM, with greater discrepancy in those with thinner IVS. There are significant differences in MV lengths measured using different imaging techniques.


Subject(s)
Cardiomyopathy, Hypertrophic , Echocardiography, Three-Dimensional , Echocardiography, Transesophageal , Mitral Valve , Multimodal Imaging , Ventricular Septum , Humans , Cardiomyopathy, Hypertrophic/surgery , Cardiomyopathy, Hypertrophic/diagnostic imaging , Middle Aged , Male , Female , Prospective Studies , Ventricular Septum/diagnostic imaging , Ventricular Septum/surgery , Mitral Valve/diagnostic imaging , Mitral Valve/surgery , Echocardiography, Transesophageal/methods , Echocardiography, Three-Dimensional/methods , Aged , Magnetic Resonance Imaging, Cine/methods , Cardiac Surgical Procedures/methods , Magnetic Resonance Imaging/methods
13.
Article in English | MEDLINE | ID: mdl-39120116

ABSTRACT

OBJECTIVES: This study aims to investigate the short-term effects of transapical beating-heart septal myectomy (TA-BSM) on left atrial (LA) anatomy and function and its association with clinical indicators in patients with hypertrophic obstructive cardiomyopathy (HOCM). METHODS: A total of 105 HOCM patients who received TA-BSM were included. Clinical and comprehensive echocardiographic data were obtained before surgery, at discharge, and 3 months after myectomy. LA reverse remodelling was defined as LA maximum volume index (LAVI) ≤34 ml/m2 and a change of ≥10%. RESULTS: At 3 months after TA-BSM, New York Heart Association (NYHA) functional class and 6-min walking test were significantly improved, N-terminal pro-B-type natriuretic peptide (NT-proBNP) decreased, left ventricular outflow tract (LVOT) peak gradient and mitral regurgitation were significantly reduced. LAVI decreased in 76%, with a median change of 20%, and the criteria for LA reverse remodelling were met in 48%. LA strain parameters were improved at 3 months after TA-BSM. Moreover, left ventricular (LV) diastolic function was significantly improved, but LV global longitudinal strain was not significantly changed at 3 months after operation. Improvement in LVOT peak gradient, LAVI, LA reservoir strain (LASr) and conduit strain (LAScd) were associated with reduction in NT-proBNP. CONCLUSIONS: Along with effectively relieving the obstruction of the LVOT and mitral regurgitation, TA-BSM could significantly improve LA size and function during the short-term follow-up for HOCM patients. The indicators of LA reverse remodelling were associated with reduction in a biomarker of myocardial wall stress, indicating the early recovery of LV relaxation and clinical status for patients.

14.
Front Cardiovasc Med ; 11: 1391047, 2024.
Article in English | MEDLINE | ID: mdl-39131704

ABSTRACT

Left bundle branch pacing (LBBP) has proven to be an alternative method for delivering physiological pacing to achieve electrical synchrony of the left ventricle (LV), especially in patients with atrioventricular block and left bundle branch block (LBBB). However, it is unclear whether it still achieved in patients whose left bundle branch (LBB) has had surgery-induced damage. The Morrow operation (Morrow septal myectomy) is regarded as one of the most effective treatments for hypertrophic obstructive cardiomyopathy (HOCM). The surgery resects small sections of muscle tissue in the proximal ventricular septum nearby or contains the LBB, which means that physical damage to the LBB is almost inevitable. Approximately 2%-12% of patients may need pacemaker implanted after Morrow surgery. LBBP is a feasible and effective method for achieving electric resynchronization of LBBB compared to right ventricular pacing (RVB). Nevertheless, there is a dearth of data on LBBP in third-degree atrioventricular block (AVB) following Morrow surgery. We report a case of successful LBBP in those patients.

15.
JACC Case Rep ; 29(15): 102409, 2024 Aug 07.
Article in English | MEDLINE | ID: mdl-39157555

ABSTRACT

A left atrium-to-left ventricle valved conduit is an alternative to conventional mitral valve (MV) replacement in patients with MV stenosis associated with a heavily calcified annulus. We describe a series of 6 patients with hypertrophic cardiomyopathy who received a valved conduit to bypass a stenotic MV, with or without concomitant septal myectomy.

16.
Strabismus ; : 1-8, 2024 Jul 30.
Article in English | MEDLINE | ID: mdl-39076154

ABSTRACT

Background: Inferior oblique muscle overaction (IOOA) is a prevalent ocular muscle motility disorder. This study aimed to investigate the outcomes and impact of IO muscle myectomy with and without tucking the proximal end of the muscle into Tenon's capsule in two surgical groups as a treatment for IOOA. Methods: Randomized cohort study that involves two surgical groups. A total of 33 patients (55 eyes). Patients were randomly divided into two groups: first group underwent myectomy without tucking the proximal end of the muscle, whereas the second group underwent myectomy with tucking of the proximal end of the muscle. Each patient had four visits. During each visit, each patient had a comprehensive ophthalmic examination. Surgical success was defined as IOOA grade range of 0 to + 1 and correction of hypertropia in primary gaze. Results: The median age was 6 years. Sixteen (48.5%) patients underwent IO muscle myectomy with tucking, while 17 (51.5%) had an IO myectomy without tucking. Both the tucking and without tucking groups showed reductions in the angles of deviations, but there was no significant difference between the groups at baseline or postoperatively at 6 months. The success rate was 92.85% for the tucking group and 92.59% for the without tucking group. Conclusion: The study suggests that both surgical techniques are effective in correcting IOOA and associated HT, without any clear benefits of the addition of tucking. Ultimately, the two surgical techniques are equally effective, and the choice of surgical technique remains a personal preference of the surgeon.


The study found that incorporating myectomy with tucking the proximal end of the muscle into Tenon's capsule does not improve the overall effectiveness of surgery. Yet, the decision to include tucking remains a personal choice for the surgeon.

17.
Article in English | MEDLINE | ID: mdl-38950769

ABSTRACT

OBJECTIVE: To characterize changes in ventricular morphology in patients with hypertrophic cardiomyopathy who develop left ventricular (LV) outflow tract obstruction. METHODS: We reviewed patients with hypertrophic cardiomyopathy with LV outflow tract obstruction who underwent septal myectomy from May 2012 to June 2023. Among 68 patients initially without obstruction documented up to 7.6 years (interquartile range, 6.3-9.4 years) before the operation, a comparison was made with 78 patients with nonobstructive hypertrophic cardiomyopathy over a similar period. Patients who did not develop obstruction were matched with those who did on sex, age, and maximum septal wall thickness during the initial echocardiography, identifying 41 matched pairs. Echocardiographic data, including 5 measures of angulation, were compared between the groups. RESULTS: The median interval between echocardiographic assessments was 7.5 years (interquartile range, 6.3-8.1 years) among patients with obstruction versus 7.3 years (interquartile range, 6.2-9.0 years) in patients without nonobstruction. Patients with obstruction were more likely to have hypertension at both times. The maximum septal wall thickness increased within both groups (both P values < .001), but the magnitude of increase was not different between groups (P = .130). Patients with obstruction exhibited a greater increase in LV mass (P < .001) compared with patients without obstruction (P = .004). Aortic angulation significantly increased in 4 of the 5 measurements (all P values < .001) in patients with obstruction, whereas patients with no obstruction showed no change. Anterior and posterior mitral valve leaflet lengths and coaptation lengths remained similar in both groups over time. CONCLUSIONS: The development of LV outflow tract obstruction in patients with hypertrophic cardiomyopathy was associated with progressive LV outflow tract angulation and increased LV hypertrophy, as reflected by LV mass. Progression to obstruction was not related to changes in the mitral valve leaflet morphology.

18.
Korean J Ophthalmol ; 38(4): 296-303, 2024 Aug.
Article in English | MEDLINE | ID: mdl-38956746

ABSTRACT

PURPOSE: Strabismus in patients with craniosynostosis is common, but surgical correction of strabismus in these patients remains challenging. We report our findings in six patients (four of whom were Korean) with craniosynostosis who underwent strabismus surgery to specifically address V-pattern horizontal strabismus with moderate-to-severe inferior oblique (IO) overaction, using IO myectomy at a single tertiary hospital between 2005 and 2016. METHODS: We recorded preoperative characteristics including sex, age, type of strabismus, versions grading, refractive error, and visual acuity. The grading of cyclorotation of horizontal rectus muscles by V-pattern categorized using coronal computed tomography imaging. RESULTS: Of the six patients, exodeviation was found in four patients and vertical deviation in two patients in primary position. One patient had both horizontal and vertical strabismus. Available computed tomography imaging showed that V-patterns were category 1 (mild) in two patients, category 2 (moderate) in one patient, and category 3 (severe) in two patients. Complete success was defined as absence of IO overaction any more. Overall complete success rate of IO myectomy was 83.3%. CONCLUSIONS: IO myectomy appeared to have some benefits in V-pattern horizontal strabismus with moderate-to-severe IO overaction in patients with craniosynostosis.


Subject(s)
Craniosynostoses , Eye Movements , Oculomotor Muscles , Ophthalmologic Surgical Procedures , Strabismus , Tomography, X-Ray Computed , Humans , Craniosynostoses/surgery , Craniosynostoses/complications , Male , Oculomotor Muscles/surgery , Oculomotor Muscles/physiopathology , Female , Ophthalmologic Surgical Procedures/methods , Strabismus/surgery , Strabismus/physiopathology , Strabismus/etiology , Retrospective Studies , Eye Movements/physiology , Child, Preschool , Treatment Outcome , Infant , Visual Acuity , Vision, Binocular/physiology , Follow-Up Studies , Child
19.
Front Cardiovasc Med ; 11: 1410222, 2024.
Article in English | MEDLINE | ID: mdl-39055661

ABSTRACT

Background: Aortic stenosis (AS) in combination with left ventricular outflow tract obstruction (LVOTO) has occasionally been reported. However, making a precise diagnosis and successfully treating this combination is challenging due to the hemodynamic interaction between the two conditions. Case summary: A 56-year-old male patient who had been diagnosed with severe AS and asymmetric left ventricular hypertrophy underwent aortic valve replacement (AVR) and a conventional septal myectomy. Immediately after the procedure, significant systolic anterior motion and mitral regurgitation developed, necessitating a surgical mitral edge-to-edge repair. Ten days after the procedure, the patient developed hematuria and LVOTO, which was confirmed by echocardiography. Because the LVOTO might have been the cause of the hematuria, the patient underwent alcohol septal ablation, but this had little effect. Three months later, a transapical beating-heart septal myectomy (TA-BSM) was performed in our hospital. Postoperatively, the LVOTO had been significantly ameliorated and the hematuria had resolved. Conclusion: For patients with AS and LVOTO due to a hypertrophic interventricular septum, inadequate amelioration of the LVOTO after AVR may lead to severe hemolytic hematuria. TA-BSM is a minimally invasive, safe, and effective surgical procedure for ameliorating LVOTO in patients with aortic valve prostheses.

20.
Rev Cardiovasc Med ; 25(1): 21, 2024 Jan.
Article in English | MEDLINE | ID: mdl-39077642

ABSTRACT

Background: Right ventricular involvement in hypertrophic cardiomyopathy is uncommon. This study aimed to evaluate clinical outcomes of the modified septal myectomy in patients diagnosed with biventricular hypertrophic cardiomyopathy (BHCM), a subject seldom explored in the literature. Methods: We conducted a retrospective cohort study from January 2019 to January 2023, enrolling 12 patients with BHCM. Each patient underwent a modified septal myectomy and was followed postoperatively. Clinical data and echocardiographic parameters, including the ventricular outflow tract peak pressure gradient and maximum interventricular septum thickness, were collected and analyzed. Results: The study cohort had a median age of 43.0 (interquartile range 14.5-63.0) years at surgery, with four patients (33.3%) being children. Two patients (16.7%) previously underwent percutaneous transluminal septal myocardial ablation. Surgical relief of biventricular outflow tract obstruction (BVOTO) was achieved in five patients (41.7%), aside from those managed solely for left ventricular outflow tract obstruction. In five instances, three-dimensional (3D) printing technology assisted in surgical planning. The postoperative interventricular septum thickness was significantly reduced (21.0 mm preoperative vs. 14.5 mm postoperative, p < 0.001), effectively eliminating residual ventricular outflow tract obstruction. There were no severe complications, such as septal perforation or third-degree atrioventricular block. During a mean follow up of 21.2 ± 15.3 months, no sudden deaths, residual outflow tract obstruction, permanent pacemaker implantation, recurrent systolic anterior motion, or reoperations were reported. Conclusions: Our findings affirm that the modified septal myectomy remains the gold standard treatment for BHCM, improving patient symptoms and quality of life. BVOTO relief can be safely and effectively achieved through septal myectomy via transaortic and pulmonary valve approaches in selected patients. For intricate cases, the application of 3D printing technology as a preoperative planning tool is advised to optimize surgical precision and safety.

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