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Journal of Cardiothoracic Surgery May 2024A 53-year-old woman with the dilated phase of hypertrophic cardiomyopathy underwent orthotopic heart transplantation. The donor heart was evaluated as normal...
A 53-year-old woman with the dilated phase of hypertrophic cardiomyopathy underwent orthotopic heart transplantation. The donor heart was evaluated as normal preoperatively without mitral regurgitation or the left atrium dilation, transplanted using the modified bicaval technique. Although the heart beat satisfactorily after aortic declamping, massive mitral regurgitation was observed without any prolapse or annular dilation. Because of the difficulty in weaning from cardiopulmonary bypass, a second aortic cross-clamp was applied, and we detached the inferior vena cava and the right side of the left atrial anastomosis to approach the mitral valve, obtaining a satisfactory exposure. No abnormalities were observed in the mitral valve leaflets, annulus or subvalvular apparatus. Subsequent in vivo mitral annuloplasty using prosthetic full ring successfully controlled the regurgitation, and the patient was easily weaned from cardiopulmonary bypass. She discharged to home with good mitral valve and cardiac functions. And the patient has been doing well without any recurrence of MR or heart failure for over a year after surgery.
Topics: Humans; Heart Transplantation; Middle Aged; Female; Mitral Valve Insufficiency; Mitral Valve; Tissue Donors; Mitral Valve Annuloplasty; Cardiomyopathy, Hypertrophic
PubMed: 38741144
DOI: 10.1186/s13019-024-02788-7 -
The International Journal of... May 2024Both Neurofibromatosis type 1 (NF1) and Noonan syndrome (NS) are RASopathies. Characteristic cardiac phenotypes of NS, including specific electrocardiographic changes,...
UNLABELLED
Both Neurofibromatosis type 1 (NF1) and Noonan syndrome (NS) are RASopathies. Characteristic cardiac phenotypes of NS, including specific electrocardiographic changes, pulmonary valve stenosis and hypertrophic cardiomyopathy have not been completely studied in NF1.
PURPOSE
The aims of this study were to assess: (1) similarities in the prevalence and types of ECG and conventional echocardiographic findings described in NS in asymptomatic patients with NF1, and (2) the presence of discrete myocardial dysfunction in NF1 patients using myocardial strain imaging.
METHODS
Fifty-eight patients with NF1 (ages 0-18 years), and thirty-one age-matched healthy controls underwent cardiac assessment including blood pressure measurements, a 12-lead ECG, and detailed echocardiography. Quantification of cardiac chamber size, mass and function were measured using conventional echocardiography. Myocardial strain parameters were assessed using 2-Dimensional (2D) Speckle tracking echocardiography.
RESULTS
Asymptomatic patients with NF1 had normal electrocardiograms, none with the typical ECG patterns described in NS. However, patients with NF1 showed significantly decreased calculated Z scores of the left ventricular internal diameter in diastole and systole, reduced left ventricular mass index and a higher incidence of cardiac abnormal findings, mainly of the mitral valve, in contrast to the frequently described types of cardiac abnormalities in NS. Peak and end systolic global circumferential strain were the only significantly reduced speckle tracking derived myocardial strain parameter.
CONCLUSIONS
Children with NF1 demonstrated more dissimilarities than similarities in the prevalence and types of ECG and conventional echocardiographic findings described in NS. The role of the abnormal myocardial strain parameter needs to be explored.
PubMed: 38739321
DOI: 10.1007/s10554-024-03125-8 -
Cureus Apr 2024The clinical presentation of inflammatory bowel disease (IBD) includes both gastrointestinal manifestations and extraintestinal manifestations (EIM). Over the past...
The clinical presentation of inflammatory bowel disease (IBD) includes both gastrointestinal manifestations and extraintestinal manifestations (EIM). Over the past years, a growing number of studies have indicated that patients suffering from IBD have an increased risk of developing cardiovascular disease. Although the precise prevalence of cardiac complications in IBD remains uncertain, emerging evidence suggests a heightened incidence compared to the general population. Valvular heart disease (VHD) in IBD encompasses calcific aortic valve disease, mitral valve prolapses, and endocarditis, potentially associated with chronic inflammation. Considering the role of inflammation in developing cardiovascular manifestations, the management should include preventing flares and maintaining remission for as long as possible. This case highlights the intricate interplay between IBD and cardiovascular complications, particularly valvular abnormalities. We present a 37-year-old male with a history of ulcerative colitis (UC) who was found to have multiple valvular diseases.
PubMed: 38738160
DOI: 10.7759/cureus.57996 -
Journal of Clinical Medicine May 2024Mitral valve prolapse (MVP) and mitral annular disjunction (MAD) are common valvular abnormalities that have been associated with ventricular arrhythmias (VA). Cardiac...
Mitral valve prolapse (MVP) and mitral annular disjunction (MAD) are common valvular abnormalities that have been associated with ventricular arrhythmias (VA). Cardiac magnetic resonance imaging (CMR) has a key role in risk stratification of VA, including assessment of late gadolinium enhancement (LGE). Single-center retrospective analysis of patients with MVP or MAD who had >1 CMR and >1 24 h Holter registration available. Data are presented in detail, including evolution of VA and presence of LGE over time. A total of twelve patients had repeated CMR and Holter registrations available, of which in four (33%) patients, it was conducted before and after minimal invasive mitral valve repair (MVR). After a median of 4.7 years, four out of eight (50%) patients without surgical intervention had new areas of LGE. New LGE was observed in the papillary muscles and the mid to basal inferolateral wall. In four patients, presenting with syncope or high-risk non-sustained ventricular tachycardia (VT), programmed ventricular stimulation was performed and in two (50%), sustained monomorphic VT was easily inducible. In two patients who underwent MVR, new LGE was observed in the basal inferolateral wall of which one presented with an increased burden of VA. In patients with MVP and MAD, repeat CMR may show new LGE in a small subset of patients, even shortly after MVR. A subgroup of patients who presented with an increase in VA burden showed new LGE upon repeat CMR. VA in patients with MVP and MAD are part of a heterogeneous spectrum that requires further investigation to establish risk stratification strategies.
PubMed: 38731198
DOI: 10.3390/jcm13092669 -
European Journal of Case Reports in... 2024Mitral valve prolapse (MVP) is a primary valvular disease of the mitral valve with a prevalence of 2.4% of the general population. Valve abnormalities range from simple...
UNLABELLED
Mitral valve prolapse (MVP) is a primary valvular disease of the mitral valve with a prevalence of 2.4% of the general population. Valve abnormalities range from simple fibroelastic deficiency of the leaflets to diffuse myxomatous degenerative changes. MVP is a usually a benign condition. However, the scattered reports of sudden cardiac death in patients with MVP in the absence of severe mitral insufficiency or coronary artery disease suggest the existence of a malignant phenotype of MVP. We report a case of a young female who survived life-threatening arrhythmias and cardiac arrest and was found to have characteristic features of the malignant phenotype of MVP and had an implantable cardioverter defibrillator as a secondary prevention.
LEARNING POINTS
Malignant MVP may be associated with life-threatening arrhythmias and sudden cardiac death.MVP is not always a benign condition, and physicians should be aware of the diagnostic criteria for malignant MVP.Echocardiography and cardiac magnetic resonance are crucial diagnostic methods to detect signs suggestive of malignant MVP.
PubMed: 38715888
DOI: 10.12890/2024_004505 -
Journal of Cardiothoracic Surgery May 2024Injury to coronary arteries during mitral surgery is a rare but life-threatening procedural complication, an anomalous origin and course of the left circumflex artery...
Injury to coronary arteries during mitral surgery is a rare but life-threatening procedural complication, an anomalous origin and course of the left circumflex artery (LCx) increase this risk. Recognizing the anomaly by the characteristic angiographic pattern and identifying its relationship with the surrounding anatomical structure using imaging techniques, mainly transesophageal echocardiography (TOE) or coronary computed tomography angiography (CCTA), is of crucial importance in setting up the best surgical strategy. We report a case of anomalous origin of a circumflex artery (LCx) from the proximal portion of the right coronary artery (RCA) with a pathway running retroaortically through the mitro-aortic space. An integrated diagnostic approach using a multidisciplinary team with a cardiologist and an imaging radiologist allowed us to decide the surgical strategy. We successfully performed a mitral valvular repair using a minimally invasive minithoracotomic approach and implanting a complete semirigid ring.
Topics: Humans; Aortic Valve; Computed Tomography Angiography; Coronary Angiography; Coronary Vessel Anomalies; Coronary Vessels; Echocardiography, Transesophageal; Mitral Valve; Mitral Valve Insufficiency
PubMed: 38715080
DOI: 10.1186/s13019-024-02779-8 -
Clinical Interventions in Aging 2024In mixed aortic valve disease (MAVD), the results of transcatheter aortic valve replacement (TAVR) are conflicting. There is limited data on the outcomes of TAVR in... (Comparative Study)
Comparative Study
PURPOSE
In mixed aortic valve disease (MAVD), the results of transcatheter aortic valve replacement (TAVR) are conflicting. There is limited data on the outcomes of TAVR in patients with bicuspid aortic valve (BAV) and MAVD. The objective of this study is to compare outcomes after TAVR in BAV patients with MAVD and predominant aortic stenosis (PAS).
PATIENTS AND METHODS
Patients with BAV who underwent TAVR between January 2016 and April 2023 were included. The primary outcome was device success. The secondary endpoints were periprocedural mortality and other complications as defined by the Valve Academic Research Consortium-3 (VARC-3). Propensity score matching was used to minimize potential confounding.
RESULTS
A total of 262 patients were included in this study, 83 of whom had MAVD. The median age was 72 years, and 55.7% were male. The baseline comorbidity risk files were comparable between the two groups. Patients with MAVD had more mitral regurgitation, tricuspid regurgitation and pulmonary hypertension, larger annular and left ventricular outflow tract dimensions, and more severe calcification than PAS. In the unmatched population, MAVD patients had similar device success rate (69.9% vs 79.9%, =0.075) and 30-day mortality (3.6% vs 3.4%, =1) compared to PAS. Propensity score matching resulted in 66 patient pairs. Device success rate were still comparable in the matched population. Other clinical outcomes, including stroke, bleeding (type 2-4), major vascular complications, acute kidney injury (stage 2-4) and permanent pacemaker implantation, were comparable between the two groups. Multivariable logistic regression analysis did not show MAVD to be an independent negative predictor of device success. At one year, survival was similar between patients with MAVD and those with PAS.
CONCLUSION
For the bicuspid valve, patients with MAVD had a more challenging anatomy. MAVD patients associated with comparable 30-day clinical outcomes after TAVR compared to PAS patients in patients with BAV.
Topics: Humans; Transcatheter Aortic Valve Replacement; Male; Female; Aortic Valve Stenosis; Aged; Bicuspid Aortic Valve Disease; Propensity Score; Aged, 80 and over; Postoperative Complications; Retrospective Studies; Treatment Outcome; Aortic Valve; Middle Aged; Risk Factors; Heart Valve Diseases
PubMed: 38711477
DOI: 10.2147/CIA.S447272 -
European Heart Journal. Cardiovascular... May 2024To explore the presence of left ventricular (LV) and left atrial (LA) morphological and functional abnormalities in patients with Barlow's disease (BD) without...
AIMS
To explore the presence of left ventricular (LV) and left atrial (LA) morphological and functional abnormalities in patients with Barlow's disease (BD) without significant mitral regurgitation (MR) and to investigate whether these abnormalities may predict MR progression.
METHODS AND RESULTS
Consecutive patients with BD were retrospectively identified from two tertiary centers; those with MR graded from trivial to mild-to-moderate were selected and matched with healthy controls in a 1:1 ratio. Conventional and speckle-tracking echocardiographic data were collected. The development of moderate-to-severe or greater MR was evaluated on follow-up echocardiograms.Patients with BD (n=231) showed increased LV dimensions and indexed LV mass (LVMi) in comparison to controls (p<0.001); LV remodeling worsened with higher MR severity and was accompanied by an increased prevalence of eccentric LV hypertrophy (eLVH). Moreover, BD patients had larger LA volumes and more impaired LA reservoir strain versus controls (p<0.001), while LV strain was similar between the two groups. Multivariable linear regression analyses in the overall population identified BD and MR grade as independent predictors of remodeling markers (LV dimensions, LVMi and LA volume), and BD as independent correlate of LA strain.MR progression was observed in 51 BD subjects (out of 170 patients with available follow-up). On Cox regression analysis, age, eLVH, mild-to-moderate MR and mitral annular disjunction (MAD) emerged as independent predictors of MR progression.
CONCLUSIONS
BD patients without significant MR show early LV and LA remodeling, together with reduced LA strain. MR progression was associated with eccentric LV remodeling, MAD, and MR severity.
PubMed: 38708450
DOI: 10.1093/ehjci/jeae121 -
Cureus Apr 2024A 72-year-old woman with recently diagnosed non-small cell lung cancer, who underwent cardiac bypass and bioprosthetic mitral valve replacement presented to our cancer...
A 72-year-old woman with recently diagnosed non-small cell lung cancer, who underwent cardiac bypass and bioprosthetic mitral valve replacement presented to our cancer center with lightheadedness, severe fatigue, and shortness of breath. Initial blood tests showed mild hemolytic anemia. The patient also complained of occasional bright red bleeding per rectum. Esophagogastroduodenoscopy and colonoscopy did not reveal an acute source of bleeding. An initial transesophageal echocardiogram did not show significant valvular or paravalvular abnormalities. Meanwhile, the patient's hemolytic anemia worsened. She received eight units of packed red blood cell transfusions. Schematic workup for hemolytic anemia revealed negative Coomb's test, positive urine hemosiderin, normal ADAMTS13 activity, and absent splenomegaly. A relook of the patient's transesophageal echocardiogram (TEE) showed a small paravalvular leak of the bioprosthetic mitral valve. The patient was referred to a tertiary center, and repair of the perivalvular leak with glue resolved her hemolytic anemia, subsequently improving the lab values, symptoms, and quality of life. This case highlights the schematic workup of hemolytic anemia and also the importance of recognizing the association between hemolytic anemia and valvular abnormalities.
PubMed: 38707129
DOI: 10.7759/cureus.57552 -
Clinical Medicine Insights. Case Reports 2024Kartagener's syndrome, a rare autosomal recessive genetic disorder, is characterized by primary ciliary dyskinesia (PCD), resulting in defective cilia function in the...
BACKGROUND
Kartagener's syndrome, a rare autosomal recessive genetic disorder, is characterized by primary ciliary dyskinesia (PCD), resulting in defective cilia function in the respiratory tract and fallopian tubes.
CASE PRESENTATION
This case report discusses a 23-year-old female with Kartagener's syndrome, bronchiectasis, and cardiac involvement, who presented with shortness of breath, cough, and syncope. Notably, she received home oxygen therapy but became exhausted, leading to loss of consciousness. Clinical examination revealed prominent heart sounds and abnormal lung findings. Laboratory results indicated leukocytosis, and an ECG confirmed dextrocardia and cardiac abnormalities. Doppler studies identified mitral and tricuspid regurgitation along with severe pulmonary arterial hypertension. Antibiotics were administered for coagulase-negative Staphylococcus infection. The patient improved with a treatment regimen, including oxygenation and nebulization. Regular follow-up and patient education were emphasized.
CONCLUSION
This case underscores the complexity of Kartagener's syndrome and the importance of a multidisciplinary approach in managing its respiratory and cardiac manifestations.
PubMed: 38706639
DOI: 10.1177/11795476241251940