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Das Journal of Thoracic and Cardiovascular Surgery
Available online March 15, 2023
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Hypertrophic cardiomyopathy (HCM) with apical left ventricular (LV) aneurysm is a phenotype associated with a fourfold increased risk of sudden cardiac death. In this study, we describe the surgical outcome of concomitant apical aneurysm repair in patients undergoing transapical myectomy for HCM.
We identified 67 patients with apical LV aneurysms who underwent transapical myectomy and apical aneurysm repair between July 2000 and August 2020. Long-term survival was compared with that of 2746 consecutive patients who underwent transaortic septomectomy for obstructive HCM with subaortic obstruction.
Transapical myectomy was indicated for midventricular obstruction (n=44) and/or LV remodeling in diastolic heart failure (n=29). Preoperatively, 74.6% (n=50) of patients had New York Heart Association class III/IV heart failure and 34.3% (n=23) of patients had syncope or pre-syncope. Atrial fibrillation was documented in 22 (32.8%) patients and episodes of ventricular arrhythmias were recorded in 30 (44.8%) patients. A thrombus was present in the apical aneurysm in 6 patients. During a median follow-up (IQR) of 4.9 years (1.8-7.6) years, the estimated 1-year and 5-year survival rates were 98.5% and 94.5%, respectively, which did not differ significantly from those of differentiated patients undergoing transaortic septomectomy in obstructive HCM (p=0.52) and general US population matched for age and sex (p=0.40).
Apical aneurysm repair in conjunction with septal myectomy is a safe procedure, and good long-term patient survival suggests that the procedure may reduce cardiovascular death in this high-risk HCM population.
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Featured Articles (6)
research article(Video) Hypertrophic Cardiomyopathy
Uncomplicated pregnancy in a patient treated with alcoholic septal ablation for obstructive hypertrophic cardiomyopathy
Canadian Journal of Cardiology, Band 33, Ausgabe 4, 2017, S. 555.e1-555.e3
Pregnancy in women with hypertrophic cardiomyopathy (HCM) is associated with a slightly increased risk of maternal morbidity and mortality and an increased risk of preterm delivery. One of the risk factors that complicate pregnancy in HCM is left ventricular outflow tract obstruction (LVOT). We present the case of a symptomatic patient with HCM and LVOS who was treated with alcoholic septal ablation and subsequently had an uncomplicated pregnancy.
In women with hypertrophic cardiomyopathy (HCM), pregnancy is associated with a slightly increased risk of maternal morbidity and mortality and an increased risk of preterm delivery. One of the factors that complicate pregnancy in these patients is the presence of dynamic left intraventricular obstruction (IVLO). We present the case of a patient with HCM and IVGOD who underwent alcoholic septal ablation and subsequently became pregnant without complications.
A natural history of apical hypertrophic cardiomyopathy with development of apical aneurysm formation: case report after a quarter century
Journal of Cardiology Cases, Band 9, Ausgabe 6, 2014, S. 221-225
We present the definitive long-term course of a man with apical hypertrophic cardiomyopathy (ApHCM) who developed an apical aneurysm at 60 years of age. At age 33, his electrocardiogram (ECG) was nearly normal. T-wave inversions were first identified in leads V5-6 of an ECG at age 36. Echocardiography first showed left ventricular (LV) lateral wall hypertrophy at 37 years of age. At the age of 42, the echocardiogram showed a diastolic shell pattern in the LV cavity, confirming the diagnosis of ApHCM. At age 54, a late systolic apical gradient of 10 mmHg (velocity: 1.6 m/s) was evident. At age 60 he developed an apical aneurysm. Computed tomography of the heart did not show significant stenosis of the main coronary arteries. Thus, in our case, LV hypertrophy and apical hypertension in CMAP promoted the formation of an apical aneurysm without coronary artery disease.
<Purpose of learning:A high apical systolic flow velocity of ≥ 1.5 m/s in apical hypertrophic cardiomyopathy (ApHCM) on echocardiography may predict the development of an apical aneurysm and prompt us to consider medical intervention with beta-blockers or verapamil to prevent aneurysm formation to delay or prevent. As recent studies have shown that ApHCM may be less benign than previously thought, seeing ApHCM without medical attention can be dangerous.>
Response of sarcoidosis associated with immune checkpoint inhibitor in the kidney: case report
Renal Medicine, 2023, article 100626(Video) Surgical treatment for hypertrophic cardiomyopathy: A historical perspective
Sarcoid-like granulomas may be a manifestation of immune-mediated adverse events associated with treatment with immune checkpoint inhibitors. To the best of our knowledge, sarcoid-like granulomas detected by renal biopsy have not been described in association with a sarcoid-like reaction associated with treatment with immune checkpoint inhibitors. We describe a 60-year-old man with renal cell carcinoma undergoing treatment with the immune checkpoint inhibitors nivolumab and ipilimumab and, 10 weeks after initiation of treatment with immune checkpoint inhibitors for the treatment of stage 3 acute renal failure, hypercalcemia and hyponatremia , was admitted to the hospital. The results of her examination showed parathyroid hormone-independent hypercalcemia (ionized calcium, 3.3 mEq/L) with an elevated 1,25-dihydroxyvitamin D level. A renal biopsy specimen showed sarcoid-like granulomas without caseation. The patient started corticosteroid therapy: 500 mg methylprednisolone bolus and continued treatment with prednisone, 80 mg once daily for the first week and then tapered off for 8 weeks. His kidney function gradually improved as the hypercalcemia subsided. After 2 weeks of treatment, her creatinine levels returned to baseline values. This case demonstrates that treatment with immune checkpoint inhibitors may be associated with renal sarcoidosis. As immune checkpoint inhibitors are increasingly used to treat cancer, clinicians must be aware of this potential inflammatory complication so that they can apply appropriate diagnostic and therapeutic approaches.
Contribution of Classical and Emerging Risk Factors to Coronary Artery Disease in Asian Indians
International Journal of Cardiology, Band 214, 2016, S. 97-106
The advantages and disadvantages of classic risk factors in coronary artery disease (CHD) are widely discussed. We analyzed the role of conventional (age, sex, diabetes, hypertension, smoking) and non-conventional (anthropometry, fasting glycemia, atherogenic plasma index - AIP, family history) risk factors in Asian Indians with CAD.
Of 11,164 subjects (4855 affected, 6309 unaffected) enrolled in the Indian Atherosclerosis Research Study (IARS), 269 unaffected subjects with abnormal ECGs and seven minors were excluded. Approximately 10,888 individuals and two subgroups, including 9,888 individuals with family history information and 1,616 individuals with an average Framingham Risk Score (FRS), were statistically analyzed using SPSS software version 17.0 and R.
A combination of classical risk factors showed good discrimination between affected and unaffected individuals (C>0.85). Hypertension (OR 3.79) or male gender (OR 5.31) were significantly associated with CAD when lipids were included or excluded from the predictive model, followed by age, diabetes and smoking. Hypertension and diabetes were more common in older patients (>55 years), while smoking was more common in younger patients (<55 years). Family history and AIP revealed a modest increase in C-score relative to classic factors (0.864 to 0.873), with a net reclassification of 7.1% in the average FRS group. The FRS classified 4% of patients with CHD as high risk, 52% were classified as patients with metabolic syndrome using the revised criteria, and over 90% had a high AIP score.
Adding PAI and family history to conventional risk factors improved risk discrimination in Asian Indians with CAD.
SMARCA4-deficient thoracic undifferentiated tumor with ALK fusion treated with alectinib achieved remarkable tumor regression: case report
JTO Clinical and Research Reports, Volume 4, Issue 4, 2023, Article 100476
Recently, SMARCA4-deficient undifferentiated tumor (SMARCA4-UT) has emerged as a distinct subtype of lung cancer. Previous studies have shown that Smarca4-UT is commonly associated with smoking-related mutations such asKRASYSTK11, rather thanEGFRoALKchanges. However, a specific precision therapy for SMARCA4-UT has not been identified. Here we report the first case of a concomitant diseaseALKSMARCA4 (BRG1) rearrangement and deficiency in a non-smoking woman with breast cancer. Alectinib was administered as first-line therapy and the patient achieved a remarkably complete response. Our case highlights the importance ofALKIdentify the rearrangement for the precise therapeutic potential of Smarca4-UT.(Video) Hypertrophic Obstructive Cardiomyopathy Animation - Treatment Procedure for HOCM Video
Variation in the treatment of fibrin sheaths associated with extracorporeal membrane oxygenation in a single service
Journal of Vascular Surgery Cases, Innovations and Techniques, Band 9, Ausgabe 2, 2023, Artikel 101121
Extracorporeal membrane oxygenation (ECMO) support for patients with cardiac or respiratory failure is increasingly used by advanced intensive care physicians. Thromboembolic complications of ECMO have been widely discussed and investigated; However, there is a lack of research and debate on the development, risks, and management of stent-associated fibrin sheaths.
Institutional Review Board approval was not required. We present three cases that describe the identification and individualized management of ECMO-associated fibrin sheaths at our institution. All three patients gave written informed consent to report their case details and imaging studies.
Of our three patients with ECMO-associated fibrin sheaths, two were successfully treated with anticoagulation alone. One was unable to receive anticoagulant therapy and an inferior vena cava filter was placed.
Fibrin sheath formation around ECMO cannulas is an unexplored complication of ECMO cannulas. We recommend an individualized approach to managing these fibrin sheaths and provide three examples of successful management.
Presentation of the meeting:American Association for Thoracic Surgery 102Dakota do NorteAnnual Meeting, Boston, MA, October 14-17, 2022
Conflict of interests:We have no conflicts of interest to disclose.
Middle panel: Magnetic resonance image of the heart showing midventricular obstruction with apical aneurysm.
Patients undergoing concomitant apical aneurysm repair and septal myectomy have equivalent long-term survival compared with an age- and sex-matched general US population.
Patients with hypertrophic cardiomyopathy complicated by an apical aneurysm are at increased risk of sudden cardiac death, ventricular arrhythmia, heart failure, and reduced survival. We found that patients who underwent apical aneurysm repair in conjunction with septomectomy had long-term survival similar to the age- and sex-matched general US population.(Video) Live Webinar: Hypertrophic Cardiomyopathy and the Surgical Treatment Apical Myectomy
Acknowledgments and Disclosures
This work was supported by the Paul family and Ruby Tsai.
© 2023 Published by Elsevier Inc. on behalf of the American Association for Thoracic Surgery