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Current Cardiology Reviews Mar 2018Mitral para-prosthetic leaks are rare but major complications of mitral heart valve replacements. When they must be re-operated, they are burdened with high mortality... (Review)
Review
BACKGROUND
Mitral para-prosthetic leaks are rare but major complications of mitral heart valve replacements. When they must be re-operated, they are burdened with high mortality rates. We proposed to review our surgical experience in terms of approach and type of operation carried out.
METHODS
Demographic, preoperative, intraoperative and postoperative characteristics of 34 patients benefited from a surgical treatment of mitral paravalvular leak, at the Brugmann University Hospital between 1996 and 2016, have been analysed retrospectively. We analysed the data to identify the risk factors of postoperative mortality. We then compared the data depending on the approach and the type of surgical treatment in order to compare the morbidity-mortality.
RESULTS
The postoperative mortality rate was 11.7%. The presence of endocarditis and increase in lactate dehydrogenase were predictive factors of mortality. Cardiac complications and acute kidney failure were significantly more common in the decease population. Direct mitral paravalvular leak suturing was more frequently performed on early apparition, anterior and isolated leaks, whereas a mitral heart valve replacement was most often performed to cure active primary endocarditis. The incidence of complications and mortality rates were identical according to the approach and the type of operation performed. A mitral para-prosthetic leak recurrence was observed in 33% of the cases.
CONCLUSION
Surgical treatment of mitral para-prosthetic leaks is accompanied by a high mortality rate. The operative strategy plays a major role and can influence the morbidity-mortality encountered in those patients.
Topics: Adult; Aged; Female; Heart Valve Prosthesis Implantation; Humans; Male; Middle Aged; Mitral Valve; Mitral Valve Insufficiency; Postoperative Complications; Prevalence; Prosthesis Failure; Retrospective Studies; Young Adult
PubMed: 29141552
DOI: 10.2174/1573403X13666171110110344 -
Ultrasound in Obstetrics & Gynecology :... Dec 2009To describe the prenatal diagnosis and review our experience of fetal congenital agenesis of the portal venous system (CAPVS) and to review the current literature on... (Review)
Review
OBJECTIVE
To describe the prenatal diagnosis and review our experience of fetal congenital agenesis of the portal venous system (CAPVS) and to review the current literature on this poorly documented vascular malformation.
METHODS
This was a retrospective survey covering the 12-year period between 1996 and 2008. The database of a single, large, ultrasonographic tertiary academic referral center in Israel was analyzed and cases with a prenatal diagnosis of CAPVS were identified. All fetuses underwent detailed biometric and structural ultrasound examinations and a precise anatomical description of the fetal umbilical, portal and hepatic venous system was noted, as well as the presence of aberrant vessels, shunt location and the presence or absence of the DV. Results of fetal echocardiography, karyotyping and toxoplasma, rubella, cytomegalovirus and herpes evaluations were determined. Medical records were evaluated. Diagnosis was confirmed by pathology, postmortem venography or neonatal ultrasound or venography. Liveborns were examined by a certified neonatologist and long-term follow-up from pediatric gastroenterology units was determined.
RESULTS
Nine cases with CAPVS were studied. In all cases an aberrant umbilical-portal vein was the primary indication for detailed portal system evaluation. Five fetuses demonstrated total CAPVS (Type I) and four showed partial agenesis of the portal vein (Type II). Among the five Type I fetuses, there was a shunt from the umbilical vein to the inferior vena cava in three (60%), to the right atrium in one and to the coronary sinus in one. In this group, in only one case could we delineate a common confluence between the splenic vein and the superior mesenteric vein shunting to the inferior vena cava. In four cases termination of pregnancy was performed due to additional findings: one case with hydrothorax, ascites and mitral atresia, one with cleft lip/palate and one with trisomy 21. One case had no additional anomalies, but the parents elected to terminate the pregnancy. All four of the Type II fetuses had a portosystemic shunt: in two cases to the right atrium, in one to the iliac vein and in one to the right hepatic vein. In three, the shunt resolved spontaneously. In only one case was abnormal liver function present over a follow-up period of 2-10 years.
CONCLUSION
CAPVS can be detected prenatally. An abnormal course of the umbilical vein necessitates prompt sonographic evaluation of the umbilical-portal venous system and meticulous investigation for additional anomalies. Complete CAPVS may be associated with remote clinical consequences of which the parents should be informed. Partial CAPVS has a favorable prognosis.
Topics: Adult; Female; Fetal Heart; Gestational Age; Humans; Israel; Portal System; Pregnancy; Retrospective Studies; Ultrasonography, Prenatal; Umbilical Veins
PubMed: 19953566
DOI: 10.1002/uog.7460 -
Journal of Echocardiography Sep 2020Hypertrophic cardiomyopathy (HCM) is the most frequent cardiac disease with genetic substrate, affecting about 0.2-0.5% of the population. While most of the patients... (Review)
Review
Hypertrophic cardiomyopathy (HCM) is the most frequent cardiac disease with genetic substrate, affecting about 0.2-0.5% of the population. While most of the patients with HCM have a relatively good prognosis, some are at increased risk of adverse events. Identifying such patients at risk is important for optimal treatment and follow-up. While clinical and electrocardiographic information plays an important role, echocardiography remains the cornerstone in assessing patients with HCM. In this review, we discuss the role of echocardiography in diagnosing HCM, the key features that differentiate HCM from other diseases and the use of echocardiography for risk stratification in this setting (risk of sudden cardiac death, heart failure, atrial fibrillation and stroke). The use of modern echocardiographic techniques (deformation imaging, 3D echocardiography) refines the diagnosis and prognostic assessment of patients with HCM. The echocardiographic data need to be integrated with clinical data and other information, including cardiac magnetic resonance, especially in challenging cases or when there is incomplete information, for the optimal management of these patients.
Topics: Asymptomatic Diseases; Atrial Fibrillation; Cardiomyopathy, Hypertrophic; Death, Sudden, Cardiac; Diagnosis, Differential; Echocardiography; Heart Failure; Humans; Mitral Valve; Prognosis; Risk Factors; Stroke Volume; Thromboembolism; Ventricular Dysfunction, Left
PubMed: 32301048
DOI: 10.1007/s12574-020-00467-9 -
British Heart Journal Mar 1968
Topics: Aortic Coarctation; Female; Heart Septal Defects, Atrial; Humans; Mitral Valve; Mitral Valve Stenosis; Transposition of Great Vessels
PubMed: 5642422
DOI: 10.1136/hrt.30.2.249 -
Journal of the American College of... Jun 2018
Topics: Antibiotic Prophylaxis; Aortic Valve; Bicuspid Aortic Valve Disease; Endocarditis; Endocarditis, Bacterial; Heart Valve Diseases; Humans; Mitral Valve Prolapse
PubMed: 29903347
DOI: 10.1016/j.jacc.2018.04.021 -
European Journal of Case Reports in... 2024Alagille syndrome (ALGS) is a multisystem disorder involving at least three systems among the liver, heart, skeleton, face, and eyes. Common cardiac associations include...
BACKGROUND
Alagille syndrome (ALGS) is a multisystem disorder involving at least three systems among the liver, heart, skeleton, face, and eyes. Common cardiac associations include pulmonary artery stenosis/atresia, atrial septal defect (ASD), ventricular septal defect (VSD) and tetralogy of fallot (ToF). Coarctation of aorta (CoA), renal and intracranial arteries are commonly involved vessels in Alagille syndrome. We present two cases with rare cardiovascular manifestations of Alagille syndrome. .
CASE 1
A 25-year-old female with a history of Alagille syndrome presented to the cardiologist office for progressive exertional dyspnoea, orthopnoea, and palpitations. She was tachycardiac on examination and had an apical diastolic rumble. A transthoracic echocardiogram (TTE) showed a left ventricular ejection fraction (LVEF) of 60% and parachute mitral valve (PMV) with severe mitral stenosis. A transoesophageal echocardiogram (TOE) showed insertion of chordae into the anterolateral papillary muscle, severe mitral stenosis with a valve area of 0.7 cm. She was referred to a congenital heart disease specialist and underwent robotic mitral valve replacement with improvement in her symptoms.
CASE 2
A 27-year-old female with known Alagille syndrome and resistant hypertension presented to the cardiologist office due to progressive exertional dyspnoea for a year. She was hypertensive and had a new 2/6 systolic ejection murmur along the left upper sternal border. TTE revealed an LVEF of 60% and pulmonary artery pressure of 19 mmHg. A CoA was suspected distal to the left subclavian artery due to a peak gradient of 38 mmHg. Cardiac magnetic resonance (CMR) imaging ruled out CoA, and diffuse narrowing of the descending thoracic aorta measuring 13-14 mm in diameter was noted. The patient was referred to a congenital heart disease specialist for further management.
CONCLUSION
PMV presenting as mitral stenosis and mid-aortic syndrome are not commonly described anomalies in association with Alagille syndrome. TTE, TOE and CMR played a key role in diagnosis and management of these patients.
LEARNING POINTS
Alagille syndrome (ALGS) is a complex multisystem disorder involving the liver, heart, skeleton, face, and eyes. Cardiovascular involvement occurs in up to 95% of the patients.Common cardiac associations include pulmonary artery stenosis/atresia, atrial septal defect (ASD), ventricular septal defect (VSD) and tetralogy of fallot (ToF).A parachute mitral valve (PMV) presenting as mitral stenosis and mid-aortic syndrome is not commonly described anomalies in association with ALGS. Here, we present such rare cases.
PubMed: 38846669
DOI: 10.12890/2024_004545 -
The Annals of Thoracic Surgery Dec 2012
Topics: Female; Heart Defects, Congenital; Heart Valve Prosthesis Implantation; Heart Ventricles; Humans; Male; Mitral Valve; Tricuspid Valve; Ventricular Function
PubMed: 23176915
DOI: 10.1016/j.athoracsur.2012.05.045 -
BMJ Case Reports Jun 2024Anomalous mitral arcade (MA) is a rare congenital anomaly. We report a case of MA in a newborn who presented with hydrops fetalis due to severe mitral regurgitation....
Anomalous mitral arcade (MA) is a rare congenital anomaly. We report a case of MA in a newborn who presented with hydrops fetalis due to severe mitral regurgitation. After birth, he developed severe respiratory failure, congestive heart failure and airway obstruction because an enlarged left atrium from severe mitral regurgitation compressed the distal left main bronchus. There is limited experience in surgical management of this condition in Thailand, and the patient's mitral valve was too small for replacement. Therefore, he was treated with medication to control heart failure and supported with positive pressure ventilation to promote growth. We have followed the patient until the current time of writing this report at the age of 2 years, and his outcome is favourable regarding heart failure symptoms, airway obstruction, growth and development. This case describes a challenging experience in the non-surgical management of MA with severe regurgitation, which presented at birth.
Topics: Humans; Mitral Valve Insufficiency; Hydrops Fetalis; Male; Infant, Newborn; Mitral Valve; Echocardiography; Heart Failure; Heart Defects, Congenital; Positive-Pressure Respiration
PubMed: 38866580
DOI: 10.1136/bcr-2023-259272 -
Interactive Cardiovascular and Thoracic... Apr 2012Partial forms of Shone complex are rare. Surgical outcomes of the complete forms have generally been poor, whereas there is a lack of data on long-term follow-up of...
Partial forms of Shone complex are rare. Surgical outcomes of the complete forms have generally been poor, whereas there is a lack of data on long-term follow-up of surgically treated adult partial complex. Between 2001 and 2011, nine patients (age: 38 ± 8 years; six males, 67%) were referred for valvular heart disease. Transthoracic and transoesophageal echocardiography was performed. Data were confirmed by intra-operative findings and reports. Patients were diagnosed as partial Shone complex and presented with mitral stenosis (MS) (45%) or mitral regurgitation (22%) or aortic regurgitation (22%). All but one patient (89%) reported previous surgery: coarctation of the aorta repair (87.5%) and aortic valvulotomy (12.5%). Redo intervention included: mitral valve replacement (25%), mitral repair (25%), aortic valve replacement (37.5%) and subvalvular aortic ridge resection (25%). One patient refused surgery. Patients surgically treated before the age of 5 (87.5%) showed favourable outcome (survival rate: 100%) and a 23.6 (± 4.6)-year follow-up free from events. The patient who underwent first intervention at the age of 50 and the patient with MS who refused surgery showed a 45 (± 7)-year follow-up free from major morbidity. Patients with partial Shone complex, properly diagnosed and treated, show favourable surgical outcome free from major clinical events.
Topics: Adult; Aortic Valve; Child, Preschool; Disease-Free Survival; Echocardiography, Doppler, Color; Echocardiography, Transesophageal; Female; Heart Defects, Congenital; Heart Valve Prosthesis Implantation; Humans; Infant; Italy; Kaplan-Meier Estimate; Male; Middle Aged; Mitral Valve; Predictive Value of Tests; Reoperation; Time Factors; Treatment Outcome; Young Adult
PubMed: 22279117
DOI: 10.1093/icvts/ivr169 -
Pediatric Cardiology 1999Abnormalities of the mitral valve (MV) or the tricuspid valve (TV) morphology and/or function in patients with functional single ventricle may result in early morbidity... (Comparative Study)
Comparative Study
Abnormalities of the mitral valve (MV) or the tricuspid valve (TV) morphology and/or function in patients with functional single ventricle may result in early morbidity and death. The purpose of this study was to determine the incidence of contralateral atrioventricular valve (AVV) pathologies in mitral valve atresia (MA) and tricuspid valve atresia (TA). We retrospectively reviewed the echocardiographic data of 50 neonates with MV and 20 with TA. Appearance of the papillary muscles, chordae tendinae, and valve leaflets was assessed. AVV regurgitation was semiquantitated by color-flow Doppler and the AVV annulus diameter was measured and indexed to body surface area. MV abnormalities were found in 9 of 20 (45%) of patients with TA. The MV was myxomatous in 9 patients, the leaflets were redundant in 5 patients, and prolapsing occurred in 4 patients. Mild regurgitation was found in 2 patients. In 18 of 20 (90%) patients MV annulus size was larger than 95% of predicted normal values. TV abnormalities were found in 12 of 50 (24%) patients with MA. The TV was myxomatous in 4 patients, prolapsing in 2, and redundant in 3, and moderate TV regurgitation was found in 3 patients. In 29 of 50 (58%) patients TV annulus size was larger than 95% of predicted normal values. Contralateral AVV abnormalities in tricuspid and mitral valve atresia are common and should be assessed carefully before surgical procedures.
Topics: Blood Flow Velocity; Echocardiography, Doppler; Female; Follow-Up Studies; Fontan Procedure; Humans; Infant, Newborn; Male; Mitral Valve; Mitral Valve Insufficiency; Pulmonary Artery; Retrospective Studies; Tricuspid Atresia; Tricuspid Valve Insufficiency
PubMed: 10089244
DOI: 10.1007/s002469900440