Severity Of Mitral Valve Disease Research Articles

Correlation between Peak Left Atrial Longitudinal Strain and The Severity of Mitral Valve Disease at Haji Adam Malik General Hospital Medan

Correlation between Peak Left Atrial Longitudinal Strain and The Severity of Mitral Valve Disease at Haji Adam Malik General Hospital Medan

Decoding calcific mitral valve disease: a novel deep learning model uncovers the role of calcium burden

Abstract Background Mitral annular calcification (MAC) is a common degenerative disease that affects older adults and causes calcium deposition in the mitral annulus, increasing the risk of mitral valve (MV) dysfunction, cardiovascular events, conduction abnormalities, and mortality. While the primary imaging modality recommended for the clinical evaluation of MAC is echocardiography, it has been reported to be found incidentally in approximately 8% of routine thoracic computed tomography (CT) scans. CT scan is a sensitive imaging modality for assessing MAC, providing the resolution necessary to understand the anatomical involvement of calcification. Research has shown that the MAC burden is associated with disease activity and progression. Still, the exact correlation between the quantitative calcium burden and the degree of severity of mitral valve disease is unclear. Purpose Our study aimed to determine the association between MAC, as assessed on CT, and significant mitral stenosis (MS) or mitral regurgitation (MR), as visualised on echocardiography. In addition, we sought to determine the quantitative MAC cutoffs that would optimally predict significant MS or MR on echocardiography. Methods A retrospective cohort study was conducted at our academic centre. Inclusion criteria were: age≥60 years, resting transthoracic echocardiogram performed between 2013-2022, non-gated chest CT performed within one year before echocardiography, and any degree of MAC documented on the echocardiography report. CT scans were requested for clinical indications unrelated to mitral valve disease. Exclusion criteria were: prior mitral valve intervention, endocarditis, and congenital mitral valve disease. Significant calcific mitral valve disease was defined as ≥moderate MR and ≥mild MS on the echocardiography report, ascertained by expert readers based on multiparametric ASE criteria. MAC volume was quantified on the multi-slice CT DICOM images using a 3D U-Net deep neural network previously trained by our group on an independent cohort. Results The cohort consisted of 1,560 unique patients with a mean age of 79 years and 61% females. The echocardiographic prevalence of significant MR and MS was 10% and 4%, respectively, for 211 affected patients. The CT-based mean MAC volume was 949 mm³ in patients with significant MR or MS, as opposed to 334 mm³ in those without (P<0.001). MAC volume >1000 mm³ was the optimal cutoff to predict significant MS (specificity 90%, sensitivity 51%, area under ROC curve 0.79) and significant MR (specificity 89%, sensitivity 21%, area under ROC curve 0.60). Adjusting for age, sex, and comorbid conditions did not affect the observed association between MAC volume and mitral valvulopathy. Conclusion A novel deep learning model for quantifying MAC volume from non-cardiac clinical CT scans was efficient in screening for calcific mitral valve disease, particularly MS, and identifying patients who may benefit from further evaluation.

Outcomes of transapical mitral valve repair with neochordae implantation.

Transapical off-pump beating-heart neochord implantation (NC) has shown encouraging early results in patients with degenerative mitral regurgitation. The aim of this study was to evaluate clinical and echocardiographic 5-year outcomes of patients who underwent NC. All patients who underwent NC at our institution from November 2013 to March 2016 were included. Indications were severe symptomatic degenerative mitral regurgitation due to leaflet prolapse/flail. Patients were classified as having favorable anatomy (FA) and unfavorable anatomy (UA) on the basis of the extent and severity of mitral valve disease. All patients underwent clinical and echocardiographic follow-up at 1, 3, 6, and 12months, and annually thereafter. Data were prospectively collected and retrospectively analyzed. Outcomes were on the basis of the Mitral Valve Academic Research Consortium guidelines. One hundred consecutive patients were included in the analysis (FA: 81%; UA: 19%). Median age was 66years (interquartile range, 58-76) and median European System for Cardiac Operative Risk Evaluation (EuroSCORE) II was 1.4% (interquartile range, 0.7-2.3). Technical and procedural success were 98% and 94%, respectively. Thirty-day mortality was 2%. Device success was 94%, 92%, and 78%, at 30days, 1-year, and 5years, respectively. Patient success at 1year was 92%. Median follow-up was 5.1years. At 5years, overall survival was 83% with no difference between FA and UA patients. Cumulative incidence of severe mitral regurgitation recurrence at 5years was 14% (95% CI, 6.5%-22.8%) in FA patients and 63% (95% CI, 39.7%-86.2%) in UA patients, respectively (P<.001). Patients with FA compared with UAhad a lower incidence of reintervention (14.7% vs 43.4%; P<.001). Transapical off-pump beating heart NC might represent an acceptable option in patients with degenerative mitral valve disease and FA.

439 TRANSIENT SEVERE MITRAL REGURGITATION IN REVERSED TAKOTSUBO SYNDROME

Abstract A 65-years old Caucasian woman was referred to the emergency department for chest pain, dyspnoea and high systolic blood pressure. In the previous days she complained a constant state of anxiety and worries due to family troubles. At the admission 12 – lead EKG showed sinus rhythm, ST-T segment depression &amp;gt;1 mm in the lateral and anterior leads (from V2 to V6). Laboratory exams showed elevated values of high sensivity troponin levels (high-sensitive troponin: 41 pg/mL – maximum laboratory cut off value: 12 pg/mL). Bedside echocardiogram showed an LVEF of 40% due to apical hyperkinesis, akynesis of the basal segments and hypokinesis of the mid segments of the left ventricle; a severe double jet MR (Fig.1); a type II diastolic dysfunction (E/A: 1,5; E/e’: 14). Several B lines (&amp;gt; 3) were present in all pulmonary regions assessed by lung POCUS. Cardiac angiography demonstrated non obstructive coronary artery disease. Left ventriculography showed an hyperdynamic apex and severe basal hypokinesis. Clinical and functional status rapidly improved. Daily EKGs were registered with a progressive resolution of the ST-T segment depression. She was discharged on the 7th days and the TTE in pre-discharge showed an LVEF of 60%, without wall motion abnormalities and complete recovery of mitral regurgitation. We report a case of reversible severe mitral regurgitation in a patient with basal ballooning TTS. Mital regurgitation is generally described in the contest of classical apical TTS. The potential mechanisms for severe MR are similar to those of acute myocardial infarction. Admission and daily echocardiographic evaluation is crucial in this subset of patients, with a tailored therapy following the severity of mitral valve disease. Fig. 1

Short-term heart rate variability in healthy dogs and dogs in various stages of degenerative mitral valve disease evaluated before pharmacotherapy

Mitral valve disease (MVD) progression is associated with autonomic nervous system (ANS) imbalance. This study hypothesized that sympathetic and parasympathetic tones would be altered with MVD progression. ANS activity was assessed among different MVD classes in untreated dogs. This cross-sectional retrospective study included 29 healthy control dogs and 71 dogs with MVD divided according to American College of Internal Medicine (ACVIM) guidelines. Heart rate variability (HRV) analyses utilised time and frequency domains and were derived from 5 min, six-lead electrocardiogram recordings of sinus rhythm or respiratory sinus arrhythmia.While all time domain-related HRV parameters decreased with disease severity, only dogs in stage C exhibited significantly lower values (P < 0.05). High frequency (HF) band values decreased more than low frequency (LF) band values as disease severity increased (P < 0.05). The LF/HF ratio also progressively increased with MVD severity. A negative correlation was observed between HRV parameters and La/Ao ratio when the entire MVD case population was pooled (P < 0.05). Both sympathetic and parasympathetic tones were altered in dogs with MVD before clinical signs were observed and prior to the development of cardiomegaly. This is the first HRV study in untreated dogs with MVD. Investigating ANS imbalances preceding subclinical left-sided cardiomegaly in dogs with MVD should be considered in future age-matched, longitudinal studies to determine the potential benefits of early vagal tone augmentation in high-risk canine patients.

Small Animal Review

Summary: Plasma atrial natriuretic peptide and troponin concentrations interpreted together are useful in assessing the severity of mitral valve disease in the dog and are useful measures to augment echocardiographic findings.

P4548The relationship between sociologic aspects and rheumatic mitral valve disease severity in an endemic region

P4548The relationship between sociologic aspects and rheumatic mitral valve disease severity in an endemic region

Correlation between serum homocysteine concentration and severity of mitral valve disease in dogs.

OBJECTIVE To measure serum homocysteine concentrations in dogs with myxomatous mitral valve disease (MMVD) and identify any association between this variable and stage of MMVD. ANIMALS 53 client-owned dogs with MMVD and 10 healthy control Beagles. PROCEDURES Dogs with MMVD were allocated to 3 groups in accordance with the staging system for chronic valvular heart disease in dogs and cats of the American College of Veterinary Internal Medicine. Blood samples were collected from all dogs, and serum homocysteine and cardiac troponin 1 concentrations were measured by enzyme immunoassay and chemiluminescence immunoassay, respectively. Analyte values were tested for associations with each other and with stage of MMVD. RESULTS A significant correlation was identified between serum homocysteine concentration and stage of MMVD. Mean ± SD concentrations were 6.72 ± 1.65 μmol/L for control dogs, 13.37 ± 4.16 μmol/L for dogs with stage B MMVD, 18.86 ± 6.73 μmol/L for dogs with stage C disease, and 28.26 ± 4.48 μmol/L for dogs with stage D disease. In addition, serum homocysteine concentration was correlated with serum cardiac troponin 1 (r = 0.34) and creatinine (r = 0.46) concentrations, systolic blood pressure (r = 0.57), and left atrium-to-aortic root ratio (r = 0.28), all of which were positively correlated with stage of MMVD. CONCLUSIONS AND CLINICAL RELEVANCE Serum homocysteine concentrations of dogs with MMVD were significantly higher than those of control dogs, and significant correlations were identified between these values and several risk factors for heart failure. Measurement of serum homocysteine concentration may be useful in the prediction of severity of disease in dogs with MMVD.

Fragmented QRS on Admission Electrocardiography Predicts Long‐Term Mortality in Patients with Non–ST‐Segment Elevation Myocardial Infarction

Early diagnosis and identification of high-risk non-ST elevation myocardial infarction (NSTEMI) is an important issue. Fragmented QRS (fQRS) complexes are defined as various RSR' patterns on 12-lead resting electrocardiography (ECG). Previous studies revealed that fQRS is related with increased ventricular arrhythmias and cardiovascular mortality. The relation between fQRS and mortality in acute coronary syndromes, mitral valve disease severity and structural heart disease has been shown in different studies. The aim of this study was to investigate relation between fQRS and long-term cardiovascular mortality in NSTEMI patients. Patients who admitted to our emergency unit and diagnosed NSTEMI between 2012 and 2013, 433 patients were included prospectively. fQRS complexes determined in 85 patients. Patients were divided into two groups according to fQRS existence. All patients evaluated for their clinical, laboratory, electrocardiographic, and echocardiographic characteristics. Angiographic features of 315 patients who underwent coronary angiography was also recorded. In-hospital, 30-day and 12-month mortality was compared between these groups. Demographic characteristics and cardiovascular risk factors were similar in both groups except hyperlipidemia. GRACE risk score was higher in patients with fQRS and positively correlated with existence of fQRS. In hospital and 30-days mortality were similar but late mortality was higher in fQRS group. Predictors of late mortality were found to be age, heart rate, male sex in addition to fQRS. We found a relation between fQRS and late mortality. Fragmented QRS may be seen as a cautionary signal for extensive myocardial damage and thereby increased long-term mortality for patients with NSTEMI.

OP-80 Fragmented QRS as a Cautionary Signal of Mortality in Non ST elevation Patients

Early diagnosis and identification of high risk non-ST elevation myocardial infarction (NSTEMI) is an important issue. Fragmented QRS (fQRS) complexes are defined as various RSR’ patterns on 12-leads resting electrocardiography (ECG). Previous studies revealed that fQRS is related with increased ventricular arrhythmias and cardiovascular mortality. The relation between fQRS and mortality in acute coronary syndromes, mitral valve disease severity and structural heart disease has been shown in different studies. The aim of this study was to investigate relation between fQRS and early (1 month) and late cardiovascular mortality (12 month) in NSTEMI patients.

NT-proBNP in the mitral valve surgery.

Prognosis and severity of mitral valve disease in patients are reflected in their natriuretic peptide levels. Patients in the upper margin of this range with severe mitral valve dysfunction also present with a range of myocardial dysfunction and symptomatic progression. We investigated whether serial pre- and immediate postoperative measurements of N-terminal probrain natriuretic peptide (NT-proBNP) can serve as surrogate markers of these surgical patients' severity status and predictors of their immediate postoperative progress. Clinical characteristics, echocardiographic indices, and preoperative and postoperative day 1, 5, 7 values of NT-proBNP were retrospectively recorded in a cohort of 75 patients who underwent mitral valve surgery. They were analyzed as a whole and separately for those suffering from severe mitral regurgitation. Correlations, multiple linear regression, logistic regression, and nonparametric receiver operating characteristic curve analyses were implemented. The patients' preoperative New York Heart Association class, presence of atrial fibrillation, and left ventricular function were strongly correlated with the preoperative NT-proBNP level. Specifically for those with severe mitral regurgitation, preoperative NT-proBNP was also correlated to their left ventricular end-diastolic diameter. NT-proBNP values increased respectively postoperatively in all patients and were related to the preoperative values, the patients' preoperative characteristics, and the operative times. Logistic regression analysis identified preoperative NT-proBNP as a predictor of postoperative optimal clinical outcome (P < 0.001). NT-proBNP is a valuable biomarker of the clinical presentation and immediate postoperative outcome in patients undergoing mitral valve surgery. The preoperative measurement of NT-proBNP can be used to predict an optimal postoperative clinical outcome.

Relationship between incidentally detected calcification of the mitral valve on 64-row multidetector computed tomography and mitral valve disease on echocardiography

BackgroundMitral valve calcification is often incidentally detected on chest computed tomography (CT) scans obtained for a variety of noncardiac indications. In this study, we evaluated the association between mitral valve calcification incidentally detected on chest CT and the presence and severity of mitral valve disease on echocardiography.MethodsOf 760 patients undergoing 64-row multidetector CT of the chest, 50 with mitral valve calcification and 100 controls were referred on for echocardiography. Calcifications of the mitral valve leaflet and annulus were assessed for length, Agatston score, and site, and were compared with echocardiographic findings.ResultsMitral valve calcification was noted in 59 (7.7%) patients on multidetector CT. Fifty of these patients were assessed by echocardiography, and 32 (64%) were found to have mitral annular calcification. Nine patients (18%) had posterior mitral valve leaflet calcification, and both mitral valve leaflet and annular calcification were detected in nine (18%) cases. Nine (18%) patients had mild, three (6%) had moderate, and one (2%) had severe mitral stenosis. None of the patients with isolated mitral annular calcification had mitral stenosis; however, all the patients with mitral stenosis showed mitral valve leaflet calcification with or without mitral annular calcification (P < 0.001). Moreover, patients with mitral stenosis had a larger mitral calcification length and greater Agatston scores in comparison with those without mitral stenosis (P = 0.001). While 31 patients (62%) with mitral calcification had mitral regurgitation on echocardiography, 21 (21%) in the control group showed mitral regurgitation (P = 0.001).ConclusionMitral valve leaflet calcification, with or without annular calcification, may be an indicator of mitral stenosis. Mitral calcification can also be considered as an indicator for mitral regurgitation in general. Therefore, patients with mitral valve calcification detected incidentally on chest CT scan may benefit from functional assessment of the valve using echocardiography.

Heritability of premature mitral valve disease in Cavalier King Charles spaniels

Mixed model analysis of 1252 records of cardiac auscultation of 4- to 5-year-old Cavalier King Charles spaniels (CKCS) from 1991 to 2008 in conjunction with the Kennel Club pedigree records of all dogs registered from the mid 1980s to September 2007 was used to estimate variance parameters of premature mitral valve disease (MVD). Data were limited to dogs ≥4 and <5 years of age to ensure diagnostic distinction between early and late onset MVD. Cardiac murmurs were detected in 108/1252 (8.6%) dogs. Heritability estimates of 0.67 (standard error, SE 0.071) for the grade of murmur and 0.33 (SE 0.072) for the presence/absence of murmur were calculated. The variance due to clinician was 0.02 (SE 0.012) for grade and 0.03 (SE 0.017) for presence/absence of murmur. These results indicate that the presence and severity of MVD, as assessed by cardiac auscultation, in 4- to 5-year-old CKCS is highly heritable and that selection against the disease should be successful.

Plasma N-terminal pro-B-type natriuretic peptide concentration helps to predict survival in dogs with symptomatic degenerative mitral valve disease regardless of and in combination with the initial clinical status at admission

Objectives Although mitral valve disease (MVD) is the most common canine heart disease, readily available prognostic markers of the disease are still lacking. The aim of this study was to evaluate the comparative ability of N-terminal pro-B-type natriuretic peptide plasma concentration (NT-proBNP) and various echocardiographic variables to predict outcome in dogs with MVD. Animals, materials and methods Seventy-four dogs with ISACHC classes 2 and 3 MVD (Groups A and B, respectively) were prospectively recruited. NT-proBNP and several echo-Doppler variables at inclusion were compared as predictors of outcome at 6 months in 54/74 dogs. Results NT-proBNP was significantly higher in Group B than in Group A ( P < 0.0001), and was the only tested variable significantly different between survivor and non-survivor dogs in both groups ( P < 0.05). In the whole canine population, a threshold of 1500 pmol/L could discriminate survivor from non-survivor dogs with a sensitivity and specificity of 80% and 73%, respectively. When combining ISACHC class with NT-proBNP levels, a cut-off of 1265 pmol/L was predictive of survival in Group A, whereas the cut-off was 2700 pmol/L for Group B. Conclusions NT-proBNP is correlated with MVD severity and could be used in combination with clinical status to predict cardiac outcome.

Association of Plasma N‐Terminal Pro‐B‐Type Natriuretic Peptide Concentration with Mitral Regurgitation Severity and Outcome in Dogs with Asymptomatic Degenerative Mitral Valve Disease

The clinical outcome of dogs affected by degenerative mitral valve disease (MVD) without overt clinical signs is still poorly defined, and criteria for identification of animals that are at a higher risk of early decompensation have not yet been determined. N-terminal pro-B-type natriuretic peptide plasma concentration (NT-proBNP) is correlated with mitral regurgitation (MR) severity and can predict disease progression in dogs with asymptomatic MVD. Seventy-two dogs with asymptomatic MVD, with or without heart enlargement (International Small Animal Cardiac Health Council: ISACHC classes 1a and 1b), and a control group of 22 dogs were prospectively recruited. Severity of MR was quantitatively assessed from the regurgitation fraction (RF) by the proximal isovelocity surface area method. Consequences of MR were evaluated from measurements of the left atrium/aorta ratio (LA/Ao), fractional shortening (FS), end-diastolic and end-systolic left ventricular volumes indexed to body surface area (EDVI and ESVI). The relevance of these echo-Doppler indices and NT-proBNP for prediction of outcome at 12 months was studied. A significant correlation was found between NT-proBNP and RF, LA/Ao, FS, and EDVI (P < .05). NT-proBNP was higher in dogs with MVD (ISACHC classes 1a and 1b) compared with the control group (P= .025 and < .001, respectively). The difference was not significant when only dogs from ISACHC class 1a with RF < 30% were considered. Lastly, NT-proBNP was higher in dogs that underwent MVD decompensation at 12 months (P < .05). NT-proBNP is correlated with MVD severity and prognosis in dogs with asymptomatic MVD.

MDCT Detection of Mitral Valve Calcification: Prevalence and Clinical Relevance Compared with Echocardiography

The purpose of this study was to analyze the prevalence and clinical significance of mitral valve calcification incidentally detected on chest CT scans in comparison with echocardiography. The data of 390 patients (227 men and 163 women; mean age, 62.4 +/- 12.2 years) who underwent MDCT of the chest and echocardiography were retrospectively evaluated. On MDCT, mitral valve leaflet and annulus calcification were visually graded on a scale of 0-3, with grade 0 denoting no calcification and grade 3 indicating severe calcification. CT findings were correlated with hemodynamic data obtained at echocardiography. Unpaired Student's t tests, chi-square analysis, and a weighted-kappa test were used to compare results. In 32 (8.2%) of 390 patients, chest MDCT revealed mitral valve leaflet calcification. Fifteen of these patients (15/390, 3.8%) presented with mitral valve stenosis. Excellent agreement (kappa = 0.882) was seen between the presence of mitral valve calcification on MDCT and echocardiographically proven mitral valve sclerosis. Mitral valve leaflet calcification on MDCT and the severity of mitral valve disease on echocardiography showed a substantial agreement (kappa = 0.730). A significant relationship was seen between the degree of mitral valve calcification on MDCT and the echocardiographically determined severity of mitral valve disease (no sclerosis vs mitral sclerosis vs mitral stenosis; p < 0.0001). Mitral valve leaflet calcification on MDCT indicates mitral valve sclerosis or stenosis. Thus, patients presenting with incidentally detected mitral valve leaflet calcification on chest CT may benefit from a functional assessment with echocardiography.

Operative Results and Outcomes in Children With Shone's Anomaly

The outcome of children with multilevel left heart obstructions with mitral valve involvement (Shone's anomaly) is generally poor. This study reviews our results and outcomes in patients with staged repair for left ventricular outflow tract obstruction (LVOTO) and mitral valve reconstruction. A total of 27 children underwent staged repair for Shone's anomaly between 1978 and 2003. The mean age at the first operation was 9.0 months (range, 2 days to 3 years). Mitral stenosis was present in all; with supravalvular mitral ring (n = 11), mitral valve abnormalities including parachute mitral valve, fused chordae, single papillary muscle (n = 10), and "typical" (Ruckman & Van Praagh) congenital mitral stenosis (n = 14). The LVOT obstruction features included subaortic stenosis (n = 16), valvar aortic stenosis (n = 7), bicuspid aortic valve (n = 24), and coarctation (n = 18). All 27 patients underwent 94 surgical procedures, including 22 mitral operations and 48 LVOT operations. There were no operative deaths at the first operation. Mean follow-up was 4.7 +/- 4.4 years (range, 6 months to 15 years). There were three late deaths (11%). All late deaths were secondary to severe mitral valve disease. Overall 15-year actuarial survival was 89%. All surviving patients are in New York Heart Association functional class I or II. Patients with Shone's anomaly are seen with a wide spectrum of major anatomic and hemodynamic abnormalities. A favorable outcome is possible for most. Operative mortality is adversely affected by the severity of mitral valve disease, the degree of left ventricular hypoplasia, and the need for multiple operative procedures.

Multiplane Transesophageal Echocardiographic Assessment of Left Atrial Appendage

Background and Objectives:Transesophageal echocardiography (TEE) allows a detailed evaluation of the structure and function of the left atrial appendage (LAA) by two-dimensional imaging and Doppler interrogation of appendage flow. LAA dysfunction has been associated with spontaneous echo contrast (SEC), thrombus formation and thromboembolism. The purpose of this study was to define normal heart reference values of LAA maximal forward velocity (LAAV), and to correlate LAAV with cardiac rhythm, mitral valve disease severity, SEC grade and left atrial thrombi development. Subjects and Methods:LAA volume measurement and pulse Doppler evaluation of LA appendage flow during TEE were undertaken in 95 subjects;32 patients with normal heart and 63 patients with various cardiac diseases. Results:Mean LAAV was 87.1±26.7 cm/s, mean LAA maximal backward velocity 66.0±15.0 cm/s and mean LAA volume 2.2±1.2 mL in those patients with normal heart (n=32). There was a negative correlation between LAAV and SEC grade, mitral stenosis severity, LA size and LAA volume. Among those patients with atrial fibrillation, LAAV was lower in the subgroup with LA thrombi than in the subgroup without thrombi (14.9±8.1 cm/s (n=16), vs. 29.7±21.2 cm/s (n=33), p<0.01). Conclusion:Reduced LAAV, SEC and mitral stenosis are all associated with LAA thrombus formation in patients with atrial fibrillation. (Korean Circulation J 2002;32(2):137-145)

Computer-controlledin vitro model of the human left heart

The decision for surgical intervention in the treatment of stenosis and for regurgitation of the mitral valve demands an objective and quantitative evaluation of the severity of mitral valve disease. The availability of ultrasound techniques capable of analysing flow velocities across valves and to produce representative images of valve orifices has increased the interest in the hydraulics of cardiac valves. To isolate and study the determinants of transmitral flow, an in vitro model of the human left heart was built. From the model it is possible to differentiate the influence of the different determinants of left heart performance on transmitral flow: preload, compliance of the left atrium and ventricle, peripheral resistance (afterload) and heart rate. The mechanical part of the model consists of a reservoir connected to an elastic closed circuit (Latex pulmonary veins, left atrium, left ventricle and aortic arch) with replaceable mitral and aortic valves. The electronic part of the model drives and controls the hydraulic part, allowing the independent regulation and monitoring of left atrial and left ventricular pressures p, volumes V and 'pV-loops' throughout the cardiac cycle at different cardiac rhythms. Left atrial filling pressure and aortic resistance are variable in a controlled fashion. Echo-Doppler study of the mitral valve and the transmitral valve flow is possible both from an atrial and a ventricular window in the model. This technical note describes the model.

Shone's anomaly: Operative results and late outcome

Shone's anomaly, a congenital cardiac malformation, consists of multiple levels of left heart obstruction including supravalvar mitral ring, parachute mitral valve, subaortic stenosis, and coarctation. The prognosis for patients with Shone's anomaly is poor. To assess operative results and late outcome, we reviewed the records of 30 consecutive patients seen with Shone's anomaly at our institution between 1966 and 1989. Anatomical diagnoses in these patients were supravalvar mitral ring (22 patients), mitral valve abnormalities including parachute mitral valve, fused chordae, or single papillary muscle (26 patients), subaortic gradients (26 patients), and coarctation (29 patients). Nineteen patients had all four lesions. Other common defects were bicuspid aortic valve (19 patients) and ventricular septal defect (20). Two patients were treated medically. The other 28 patients required 84 operative procedures with 18 patients undergoing more than one procedure. Operations included coarctation repair (28 patients), mitral valve repair or replacement (11), ventricular septal defect closure (8), subaortic resection (8), and complex left ventricular outflow tract reconstruction or bypass (4). Age at first operition ranged from 7 days to 7 years (median age, 3 months). There were no operative deaths at the first operation. However, mortality rose to 24% (4/17) after the second operation. All operative deaths were secondary to severe mitral valve disease. The survivors have been followed from 1 to 16 years (mean follow-up, 6 ± 1 years). There were no late or sudden deaths. Morbidity has included stroke (1), gastrointestinal bleeding (2), permanent heart block (1), and persistent congestive heart failure (6). We conclude that although patients with Shone's anomaly are seen with a wide spectrum of major anatomical and hemodynamic abnormalities, a satisfactory long-term outcome is possible for most. Operative mortality is adversely affected by the severity of mitral valve disease and the need for multiple operative procedures.