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European Heart Journal. Case Reports Jun 2024The coexistence of rheumatic heart disease (RHD) and pulmonary arteriovenous malformation (PAVM) is a rare clinical scenario that poses diagnostic and therapeutic...
BACKGROUND
The coexistence of rheumatic heart disease (RHD) and pulmonary arteriovenous malformation (PAVM) is a rare clinical scenario that poses diagnostic and therapeutic challenges. This case report explores the clinical presentation, diagnostic journey, and multidisciplinary management of a patient presenting with both conditions.
CASE SUMMARY
A 47-year-old female with a history of RHD presented with symptoms of dyspnoea on exertion and cyanosis, suggestive of both cardiac involvement and pulmonary involvement. Subsequent investigations involving imaging, echocardiography, and invasive pulmonary angiography revealed the coexistence of RHD and multiple PAVM in the patient's left lower lobe of the lung. The patient underwent a tailored treatment plan, initially involving percutaneous mitral balloon valvuloplasty for RHD, followed by a staged procedure of transcatheter PAVM closure with Amplatzer™ Vascular Plug II performed 1 month later. Her saturation normalized following the intervention. The patient's progress was monitored closely, with adjustments made to the treatment plan based on evolving clinical scenarios. The patient remained well in short-term follow-up.
DISCUSSION
This case highlights the complexity of managing patients having two diverse conditions RHD and PAVM coexisting together, thus emphasizing the importance of a multidisciplinary approach. The unique intersection of cardiac and pulmonary pathologies necessitates careful consideration of diagnostic nuances and tailored treatment strategies. Lessons learned from this case offer valuable insights for clinicians encountering similar scenarios and underscore the significance of individualized, patient-centred care in optimizing outcomes for those with dual pathologies.
PubMed: 38845808
DOI: 10.1093/ehjcr/ytae239 -
World Journal of Cardiology May 2024Mitral valvuloplasty using artificial chordae tendineae represents an effective surgical approach for treating mitral regurgitation. Achieving precise measurements of...
BACKGROUND
Mitral valvuloplasty using artificial chordae tendineae represents an effective surgical approach for treating mitral regurgitation. Achieving precise measurements of artificial chordae tendineae length (CL) is an important factor in the procedure; however, no objective index currently exists to facilitate this measurement. Therefore, preoperative assessment of CL is critical for surgical planning and support. Four-dimensional x-ray micro-computed tomography (4D-CT) may be useful for accurate CL measurement considering that it allows for dynamic three-dimensional (3D) evaluation compared to that with transthoracic echocardiography, a conventional inspection method.
AIM
To investigate the behavior and length of mitral chordae tendineae during systole using 4D-CT.
METHODS
Eleven adults aged > 70 years without mitral valve disease were evaluated. A 64-slice CT scanner was used to capture 20 phases in the cardiac cycle in electrocardiographic synchronization. The length of the primary chordae tendineae was measured from early systole to early diastole using the 3D image. The primary chordae tendineae originating from the anterior papillary muscle and attached to the A1-2 region and those from the posterior papillary muscle and attached to the A2-3 region were designated as cA and cP, respectively. The behavior and maximum lengths [cA (ma), cP (max)] were compared, and the correlation with body surface area (BSA) was evaluated.
RESULTS
In all cases, the mitral anterior leaflet chordae tendineae could be measured. In most cases, the cA and cP chordae tendineae could be measured visually. The mean cA (max) and cP (max) were 20.2 mm ± 1.95 mm and 23.5 mm ± 4.06 mm, respectively. cP (max) was significantly longer. The correlation coefficients (r) with BSA were 0.60 and 0.78 for cA (max) and cP (max), respectively. Both cA and cP exhibited constant variation in CL during systole, with a maximum 1.16-fold increase in cA and a 1.23-fold increase in cP from early to mid-systole. For cP, CL reached a plateau at 15% and remained elongated until end-systole, whereas for cA, after peaking at 15%, CL shortened slightly and then moved toward its peak again as end-systole approached.
CONCLUSION
The study suggests that 4D-CT is a valuable tool for accurate measurement of both the length and behavior of chordae tendineae within the anterior leaflet of the mitral valve.
PubMed: 38817650
DOI: 10.4330/wjc.v16.i5.274 -
Brazilian Journal of Cardiovascular... May 2024Female, seven years old, referred to our service complaining about congestive heart failure symptoms due to mitral valve regurgitation and atrial septal defect....
CLINICAL DATA
Female, seven years old, referred to our service complaining about congestive heart failure symptoms due to mitral valve regurgitation and atrial septal defect. Technical description: Echocardiographic findings compatible with Barlow's disease and atrial septal defect, ostium secundum type.
OPERATION
She was submitted to mitral valvuloplasty with chordal shortening and prosthetic posterior ring (Gregori-Braile®) along with patch atrioseptoplasty.
COMMENTS
Mitral valve regurgitation is a rare congenital heart disease and Barlow's disease is probably rarer. Mitral valve repair is the treatment of choice.
Topics: Humans; Heart Septal Defects, Atrial; Female; Child; Mitral Valve Insufficiency; Echocardiography; Mitral Valve Prolapse
PubMed: 38748990
DOI: 10.21470/1678-9741-2023-0278 -
Cureus Apr 2024We report a case of severe mitral stenosis (MS) in a 58-year-old female from Guyana. Though rheumatic MS continues to be less prevalent in third-world countries, it...
We report a case of severe mitral stenosis (MS) in a 58-year-old female from Guyana. Though rheumatic MS continues to be less prevalent in third-world countries, it poses a significant threat as far as morbidity and mortality are concerned. The modern definition of "Third World" is used to classify countries that are poor or developing. Countries that are part of the "third world" are generally characterized by (1) high rates of poverty, (2) economic and/or political instability, and (3) high mortality. The standard method of diagnosing MS in patients has been established as transthoracic echocardiograms (TTE), along with pertinent historical and physical exam findings. Specifically, with TTE, criteria include a mitral valve area ≤1.5 cm, severe left atrial enlargement, and elevated pulmonary artery systolic pressure >50 millimeters of mercury (mmHg). Once diagnosed with severe MS, treatment options for patients range from non-surgical percutaneous mitral balloon valvuloplasty to surgical mitral valve commissurotomy. In our case, she was a 58-year-old female with a past medical history of seizures of unknown etiology, not on any home medication regimen, presenting to the emergency department with shortness of breath, malaise, weight loss, and bilateral lower leg edema. Vitals were significant for tachycardia at 153 bpm, tachypnea at 24 breaths per minute, and saturating at 96% on room air. On the physical exam, there was an irregularly irregular rhythm, bilateral crackles at the bases, right upper quadrant tenderness to palpation, bilateral pitting edema, and no calf tenderness. Lab findings were significant for elevated brain natriuretic peptide, but three electrocardiograms were performed in the emergency department, all confirming the new onset of atrial fibrillation. A CT angiogram of the chest was performed, which ruled out pulmonary embolus but additionally found marked reflux of contrast noted within the inferior vena cava and hepatic veins, along with right atrial dilation reflective of right heart strain; additionally, mitral valve calcifications were noted. The cardiologist on duty confirmed the diagnosis using point-of-care ultrasound (POCUS) followed by TTE; the patient was rapidly transferred to a hospital with appropriate services for surgical management within the same day of arrival at the emergency department. This case highlights the importance of bedside POCUS as an additional diagnostic tool for cardiologists, along with pertinent history, physical examination findings, and laboratory findings. Proper utilization of POCUS can allow for the immediate diagnosis of severe pathologies and prevent the delay of appropriate treatment, as seen in our case. Wider adoption of POCUS practices as a part of the general initial evaluation of patients has not yet been recommended by the American Heart Association but can offer clinical benefit in morbidity/mortality with expedited progression to appropriate treatment.
PubMed: 38706999
DOI: 10.7759/cureus.57627 -
Texas Heart Institute Journal Apr 2024Klippel-Feil syndrome, characterized by congenital fusion of any 2 or more cervical vertebrae, is a rare disorder in which skeletal and other organ system-related...
Klippel-Feil syndrome, characterized by congenital fusion of any 2 or more cervical vertebrae, is a rare disorder in which skeletal and other organ system-related abnormalities have been reported. This article reports a case of mitral valve regurgitation in a patient with Klippel-Feil syndrome and related thoracic deformity who underwent mitral valvuloplasty. Postoperatively, the mitral valve regurgitation disappeared, and there has been no recurrence for 3 years. This case highlights mitral valvuloplasty via median sternotomy as an excellent treatment for mitral valve regurgitation in a patient with thoracic deformity related to Klippel-Feil syndrome.
Topics: Adult; Humans; Male; Balloon Valvuloplasty; Klippel-Feil Syndrome; Mitral Valve; Mitral Valve Insufficiency; Sternotomy; Tomography, X-Ray Computed; Treatment Outcome
PubMed: 38665003
DOI: 10.14503/THIJ-23-8282 -
Endocrine Journal Apr 2024Cardiovascular disease is one of the most important complications in girls and women with Turner syndrome (TS). Although the latest international guideline provides...
Cardiovascular disease is one of the most important complications in girls and women with Turner syndrome (TS). Although the latest international guideline provides useful suggestions for the management of cardiovascular diseases in TS, some unknown cardiac conditions warrant physicians' attention and awareness. Here, we have reported two adult cases wherein significant cardiovascular diseases were detected during the transition period. The first case patient was diagnosed with aortic crank deformity and left subclavian artery aneurysm at 14 years based on the report of cardiac catheterization, computed tomography angiography, and cardiac magnetic resonance imaging, which had remained undetected by annual evaluations using transthoracic echocardiography (TTE). This case emphasizes the importance of cardiac reevaluation during the transition period. The second case patient was diagnosed with moderate mitral valve regurgitation (MR) due to mitral valve prolapse at 18 years through TTE, although the first evaluation at 7 years by TTE detected slight MR without any clinical concerns. The condition however progressed to severe MR at 28 years, requiring mitral valvuloplasty. MR is the most common valve disease worldwide, which makes it challenging to comprehend whether the condition is a complication. However, the condition requiring surgery at this age is extremely rare, which implies the possibility of early progression. Because almost all literature on cardiovascular complications in TS is cross-sectional, further information about longitudinal cardiovascular conditions is vital for optimal care for girls and women with TS. The two cases reported in this article provide significant information for improving lifelong cardiovascular health issues in TS.
PubMed: 38658359
DOI: 10.1507/endocrj.EJ24-0038 -
Frontiers in Cardiovascular Medicine 2024Here we report a rare morphology of a cardiac fibroma in a child. A 2-year and 8-month-old toddler came for "chronic constipation" and was found to have a heart murmur...
Here we report a rare morphology of a cardiac fibroma in a child. A 2-year and 8-month-old toddler came for "chronic constipation" and was found to have a heart murmur on cardiac auscultation. Further transthoracic echocardiography suggested "a strong echogenic mass in the left ventricular wall, with some part of "a string of beads" in shape extending into left ventricle outflow tract", which was atypical for either a tumor, thrombus or vegetation. The child underwent resection of the mass and mitral valvuloplasty. Pathological examination confirmed the mass as a cardiac fibroma.
PubMed: 38606376
DOI: 10.3389/fcvm.2024.1357747 -
International Journal of Cardiology.... Apr 2024Efficacy of balloon mitral valvuloplasty (BMV) in low gradient severe rheumatic mitral stenosis (MS) is not very well defined. This study was undertaken to evaluate the...
BACKGROUND
Efficacy of balloon mitral valvuloplasty (BMV) in low gradient severe rheumatic mitral stenosis (MS) is not very well defined. This study was undertaken to evaluate the outcomes of BMV in low gradient severe rheumatic MS.
METHODS
Severe MS was defined as mitral valve area < 1.5 cm. Low gradient was defined as mean diastolic -mitral gradient (MG) < 10 mmHg and low flow as stroke volume index < 35 ml/m on echocardiography. Sixty patients were divided into normal-flow/low-gradient (NFLG) (40) and low-flow/low-gradient (LFLG) (20) groups. Post-BMV parameters were recorded after 72 h and at the end of one year.
RESULTS
Mean age was 36.2 ± 6.6 years in NFLG group and 40.6 ± 2.6 years in LFLG group (p < 0.01) and females were 75 % (n = 30) in NFLG group as compared to 60 % (n = 12) in LFLG group. Patients in the LFLG group had higher Wilkins score (p < 0.02) and prevalence of atrial fibrillation (n = 8, 40 %) as compared to NFLG group (n = 7, 17.5 %; p < 0.01). A greater decrease in MG was observed in NFLG group (p < 0.01), whereas increase in MVA was comparable in both the groups (p > 0.05). Ninety percent (n = 36) patients improved in NFLG group in comparison to 70 % (n = 14) in LFLG group (p < 0.01). At the end of one-year, symptomatic improvement persisted in all patients who became asymptomatic post-BMV.
CONCLUSION
Symptomatic improvement following BMV was better seen in NFLG group because of greater decrease in MG in comparison to LFLG group. Results of BMV were suboptimal in LFLG group because of higher sub-valvular obstruction, increased age and higher prevalence of AF.
PubMed: 38560513
DOI: 10.1016/j.ijcha.2024.101394 -
JACC. Case Reports Mar 2024A 55-year-old man submitted to emergency surgery due to cardiac perforation by stabbing. One month later, he presented with chest pain, and a transthoracic...
A 55-year-old man submitted to emergency surgery due to cardiac perforation by stabbing. One month later, he presented with chest pain, and a transthoracic echocardiogram revealed moderate-severe mitral regurgitation. After 6 months, a new transthoracic echocardiogram showed a left ventricular pseudoaneurysm, being later submitted to pseudoaneurysm exclusion and mitral valvuloplasty.
PubMed: 38549847
DOI: 10.1016/j.jaccas.2024.102240 -
CASE (Philadelphia, Pa.) Mar 2024• Congenital MS is a rare cardiac disease in canines. • BMV may improve the mobility of stenotic mitral leaflets. • AF and LA thrombi may occur in congenital MS.
• Congenital MS is a rare cardiac disease in canines. • BMV may improve the mobility of stenotic mitral leaflets. • AF and LA thrombi may occur in congenital MS.
PubMed: 38524966
DOI: 10.1016/j.case.2023.12.018