A DNA methylation atlas of normal human cell types.

DNA methylation is a fundamental epigenetic mark that governs gene expression and chromatin organization, thus providing a window into cellular identity and developmental processes1. Current datasets typically include only a fraction of methylation sites and are often based either on cell lines that underwent massive changes in culture or on tissues containing unspecified mixtures of cells2-5. Here we describe a human methylome atlas, based on deep whole-genome bisulfite sequencing, allowing fragment-level analysis across thousands of unique markers for 39 cell types sorted from 205 healthy tissue samples. Replicates of the same cell type are more than 99.5% identical, demonstrating the robustness of cell identity programmes to environmental perturbation. Unsupervised clustering of the atlas recapitulates key elements of tissue ontogeny and identifies methylation patterns retained since embryonic development. Loci uniquely unmethylated in an individual cell type often reside in transcriptional enhancers and contain DNA binding sites for tissue-specific transcriptional regulators. Uniquely hypermethylated loci are rare and are enriched for CpG islands, Polycomb targets and CTCF binding sites, suggesting a new role in shaping cell-type-specific chromatin looping. The atlas provides an essential resource for study of gene regulation and disease-associated genetic variants, and a wealth of potential tissue-specific biomarkers for use in liquid biopsies.

Post-operative anterior diaphragmatic hernias in children with Trisomy 21 after cardiac surgery.

Morgagni hernias account for less than 5% of congenital diaphragmatic hernias. They are characteristically retrosternal and bilateral, with right-sided predominance. An association between Trisomy 21 and diaphragmatic hernias resembling Morgagni hernia has been reported, but the effect of cardiac surgery on its formation has not been investigated. The purpose of this study was to determine whether there is a higher incidence of anterior diaphragmatic hernias in children with Trisomy 21 after cardiac surgery. We compared the prevalence of anterior diaphragmatic hernias in 92 patients with Trisomy 21 who underwent cardiac surgery with its prevalence in 100 children without Trisomy 21 who underwent cardiac surgery. All available CXRs of all children underwent revision for the presence of an anterior diaphragmatic hernia by a pediatric radiologist. Within the study group, four cases of an anterior diaphragmatic hernia were detected, all upon presentation to the emergency room due to breathing difficulties. No cases of an anterior diaphragmatic hernia were found in the control group (P = 0.0094). CONCLUSIONS: A high index of suspicion for an anterior diaphragmatic hernia should be maintained in children with Trisomy 21 who have undergone cardiac surgery and present with breathing difficulty. If CXR findings are uncertain, UGI series and\or CT should be performed. In light of our findings, the surgical technique has been modified in patients with DS in our medical center. WHAT IS KNOWN: • Several studies reported an association between Trisomy 21 and diaphragmatic hernia resembling Morgagni hernia, but the effect of cardiac surgery on its formation has not been investigated. WHAT IS NEW: • There is a higher incidence of anterior diaphragmatic hernia resembling a Morgagni hernia in children with Trisomy 21 after cardiac surgery. • A high index of suspicion for an anterior diaphragmatic hernia should be maintained in children with Trisomy 21 who have undergone cardiac surgery and present with breathing difficulty. If CXR findings are uncertain, UGI series and\or CT should be performed.

Long-Term Aortic Valve Function in Patients With or Without Surgical Treatment for Discrete Subaortic Stenosis.

The purpose of this study was to evaluate the long-term function of the aortic valve in patients with discrete subaortic stenosis (DSS), and its prognostic implications. 34 patients with the diagnosis of isolated DSS were treated at our pediatric heart center between 1992 and 2019. Demographic, echocardiographic and surgical data were analyzed. 26 patients had surgery to remove the subaortic membrane. The primary endpoint of this study was a change in the aortic valve function, secondary outcomes included functional class, aortic valve replacement, and reoperation due to recurrence of DSS. The mean post-operative follow-up time was 7.07 years (1-22.5). At the time of the most recent follow up, in the surgical group 11 patients (46%) had echocardiographic evidence of deterioration in their aortic valve insufficiency (AI) (p < 0.002). The deterioration was associated with a pre-operative left ventricular outflow tract (LVOT) gradient greater than 49 mmHg (p < 0.022). 8 patients with DSS were followed for a mean of 12.9 years (6.8-21.3), without meeting the criteria for surgical intervention, none showed any change in the aortic valve function during follow-up time. DSS resection may not prevent worsening of aortic valve insufficiency over time. Patients with stable AI and low LVOT gradient may not require surgery. Given these findings, it may be appropriate to carefully follow patients with new onset AI and low LVOT gradient, delaying surgical intervention.

Valve-sparing aortic root replacement for patients with a Fontan circulation.

BACKGROUND AND AIM OF THE STUDY: Aortic root aneurysm is an emerging disease related to the treatment of patients with hypoplastic left heart syndrome (HLHS) and other single-ventricle physiology. The authors' experience with valve-sparing aortic root replacement and concomitant procedures in patients with a single ventricle was reviewed. METHODS: Four patients aged 11 to 19 years presented with progressive aortic root dilatation. All had undergone a Fontan completion: two patients for HLHS, one patient for tricuspid valve atresia, and one for pulmonary atresia with a ventricular septal defect. Two patients developed more than grade 2+ aortic valve insufficiency, and one patient developed symptomatic left anterior descending coronary artery compression. The preoperative root dimension ranged from 38 to 56 mm (mean 45mm). A valve-sparing aortic root replacement, annular reduction and ascending aorta replacement was performed in all patients. Two patients underwent a Fontan revision, two an aortic valve repair, and one patient a tricuspid valve repair. RESULTS: At a mean follow up of 2.6 years, all four patients were in NYHA functional class I. Two patients underwent neo-aortic valve replacement at about two years after aortic root replacement and valve repair, as they had developed severe neo-aortic valve insufficiency. Both of these patients had HLHS. In the other two patients, the most recent echocardiography demonstrated grade 0 to 1+ aortic insufficiency, with good function of the single ventricle. CONCLUSION: Aortic root reimplantation is an effective treatment for aortic root dilatation in patients with a single ventricle. However, if associated with significant aortic insufficiency, then a long-term freedom from valve replacement is questionable.

Outcomes of sublobar resection vs lobectomy for invasive clinical stage T1N0 non-small-cell lung cancer: A propensity-match analysis.

BACKGROUND: The role of sub lobar resection (SLR; either segmentectomy or wedge resection) vs lobectomy (LBCT) for invasive clinical stage T1N0 non-small-cell-lung-cancer (NSCLC) has not been fully established yet. AIM: We aimed to characterize the preoperative parameters leading to selecting SLR and compare the overall survival (OS) and disease-free survival (DFS) of these two surgical approaches. METHODS: Clinical data on 162 patients (LBCT-107; SLR-55) were prospectively entered in our departmental database. Preoperative parameters associated with the performance of SLR were identified using univariate and multivariate cox regression analysis. The Kaplan-Meier method was used to compute OS and DFS. Comparison between LBCT and SLR groups and 32 propensity-matched groups was performed using Log-rank test. RESULTS: Median follow-up time for the LBCT and SLR groups was 4.76 (Inter-quartile range [IQR] 2.96 to 8.23) and 3.38 (IQR 2.9 to 6.19) years respectively. OS and DFS rates were similar between the two groups in the entire cohort (OS-LBCT vs SLR P = .853, DSF-LBCT vs SLR P = .653) and after propensity matching (OS-LBCT vs SLR P = .563 DSF-LBCT vs SLR P = .632). Specifically, Two- and five-year OS rates for LBCT and SLR were 90.6.% vs 92.7%, 71.8% vs 75.9% respectively. Independent predictors of selecting for SLR included older age (P < .001), reduced FEV1% (P = .026), smaller tumor size (P = .025), smaller invasive component (P = .021) and higher American Society of Anesthesiology scores (P = .014). CONCLUSIONS: In 162 consecutive and 32 matched cases, SLR and lobar resection had similar overall and disease-free survival rates. SLR may be considered as a reasonable oncological procedure in carefully selected T1N0 NSCLC patients that present with multiple comorbidities and relatively small tumors.

Senning With Aortic Translocation and Anatomic Repair for Congenitally Corrected Transposition.

BACKGROUND: Anatomic repair for congenitally corrected transposition of the great arteries with ventricular septal defect (VSD) and pulmonic stenosis has been accomplished with atrial switch and Rastelli. Aortic translocation offers a direct left ventricular outflow without an extraanatomic right ventricular-to-pulmonary conduit, which may lead to decreased reoperations. We reviewed our entire experience performing Senning with aortic translocation (SAT). METHODS: From 2007 to 2017, 8 patients (mean age, 14.1 months; size, 8.86 kg) underwent SAT. Associated anomalies included situs inversus (n = 2), dextrocardia (n = 6), multiple muscular VSDs (n = 2), abnormal or straddling atrioventricular valve chords (n = 5), and branch pulmonary artery stenosis (n = 3). Four of 8 had previous systemic arterial shunts. Mean cardiopulmonary bypass was 487 minutes, and mean cardiac ischemic time was 307 minutes. Additional procedures included repair of branch pulmonary artery stenoses and closure of multiple muscular VSDs. RESULTS: There was no hospital death. One patient was supported with extracorporeal membrane oxygenation because of junctional tachycardia on postoperative day 5. One patient required pacemaker placement for first-degree heart block. Median hospital length of stay was 31 days. Mean length of follow-up was 52 months. All patients remain well with mild or no aortic regurgitation. The first patient underwent a repeat surgical operation for pulmonary venous baffle obstruction 2 years after SAT. CONCLUSIONS: Despite the technical complexity, patient outcomes have been satisfactory. We believe SAT provides a superior anatomic repair in these complex defects. Longer-term follow-up is needed regarding late intervention.

The Relationship Between Body Mass Index and In-Hospital Mortality in Patients Following Coronary Artery Bypass Grafting Surgery.

Background: The association between Body Mass Index (BMI) and clinical outcomes following coronary artery bypass grafting (CABG) remains controversial. Our objective was to investigate the real-world relationship between BMI and in-hospital clinical course and mortality, in patients who underwent CABG. Methods: A sampled cohort of patients who underwent CABG between October 2015 and December 2016 was identified in the National Inpatient Sample (NIS) database. Outcomes of interest included in-hospital mortality, peri-procedural complications and length of stay. Patients were divided into 6 BMI (kg/m2) subgroups; (1) under-weight ≤19, (2) normal-weight 20-25, (3) over-weight 26-30, (4) obese I 31-35, (5) obese II 36-39, and (6) extremely obese ≥40. Multivariable logistic regression model was used to identify predictors of in-hospital mortality. Linear regression model was used to identify predictors of length of stay (LOS). Results: An estimated total of 48,710 hospitalizations for CABG across the U.S. were analyzed. The crude data showed a U-shaped relationship between BMI and study population outcomes with higher mortality and longer LOS in patients with BMI ≤ 19 kg/m2 and in patients with BMI ≥40 kg/m2 compared to patients with BMI 20-39 kg/m2. In the multivariable regression model, BMI subgroups of ≤19 kg/m2 and ≥40 kg/m2 were found to be independent predictors of mortality. Conclusions: A complex, U-shaped relationship between BMI and mortality was documented, confirming the "obesity paradox" in the real-world setting, in patients hospitalized for CABG.

Midterm outcomes of right ventricular outflow tract reconstruction using the Freestyle xenograft.

OBJECTIVE: Various options exist for right ventricular outflow tract (RVOT) reconstruction in congenital heart disease. The Freestyle porcine aortic root may be used but its longevity is not well defined. DESIGN: We performed a retrospective review of all non-Ross RVOT reconstructions using the Freestyle root in our institution. Survival and reintervention, either by surgery, transcatheter valve implantation, balloon valvuloplasty, or bare metal stent placement, were recorded. Factors associated with reintervention were assessed using Cox regression. RESULTS: Between January 2002 and December 2015, there were 182 patients identified. Sixteen patients were lost to follow-up and 3 patients died, unrelated to cardiac surgery. Of the remaining 163 patients, the median age was 12.2 years (interquartile range 6.4-16.4), median weight was 39.0 kg (interquartile range 19.9-59.3), and the median body surface area was 1.23 m2 (interquartile range 0.79-1.64). Ninety-three (57%) patients had tetralogy of Fallot. The median follow-up was 5.4 years (interquartile range 2.9-8 years). There were no operative or cardiac-related deaths. Thirty-eight patients (23%) required reintervention. The rate of freedom from reintervention was 93.2% (95% CI 86.7%-96.6%) at 5 years and 48.4% (95% CI 34.9%-60.6%) at 10 years. Age < 10 years, weight < 39 kg, and body surface area <1.2 m2 at the time of valve placement, as well as valve size ≤25 mm were significantly associated with need for earlier reintervention. CONCLUSIONS: The Freestyle root in the RVOT is associated with excellent survival and low midterm need for reintervention. Its longevity is comparable to published data on homografts and other bioprosthetic valves.

Surgical Treatment of Palestinian Patients With Congenital Heart Disease in a Medical Center in Israel: Challenges and Outcome.

BACKGROUND: The treatment of congenital heart disease patients in the West Bank and Gaza involves both medical and political challenges. Understanding the difficulties faced in treating the Palestinian population is an important step to improving surgical care, better allocating resources and overcoming the region's unique problems. METHODS: The Hadassah Medical Center congenital heart disease database over the 2011-2017 period was analyzed. There were 872 operations performed in patients with Israeli health insurance and 207 operations in Palestinian patients. Patient characteristics and surgical outcome were compared between the two groups using standard statistical practices. FINDINGS: The Society of Thoracic Surgeons Complexity Scores were significantly higher in the Palestinian patients, p = 0.003 (d = 0.27, 95% CI, 0.12 to 0.42). Israeli neonates had surgery at an average age of 9.5 ±â€¯7.8 days as compared to Palestinian neonates with an average age of 15.7 ±â€¯8.2 days, p < 0.001 (d = 0.78, 95% CI, 0.41 to 1.15), a finding indicative of a possible delay of treatment. Overall in hospital mortality was not significantly different. Late mortality was significantly higher for the Palestinian 5.4% (9/168) compared to Israeli patients 2% (14/698), p = 0.015 (RR = 2.67, 95% CI, 1.18 to 6.07). INTERPRETATION: The findings suggest that Palestinian patients receive later treatment and poorer follow-up care than Israeli patients. Despite the political challenges in the region surgical results are excellent and comparable between the two groups. The challenges described are not unique to congenital heart disease and may affect many medical fields. We believe that extensive collaborations between Israeli and Palestinian physicians may be key to improving the Palestinian medical care. FUNDING: None.

Repeat right ventricular outflow tract reconstruction using the Medtronic Freestyle porcine aortic root.

BACKGROUND AND AIM OF THE STUDY: A variety of valve substitutes are used for right ventricular outflow tract (RVOT) reconstruction in children and young adults after previous RVOT surgery that has led to significant pulmonary insufficiency and/or stenosis. Herein, the authors' experience with pulmonary valve replacement (PVR) using a porcine valved conduit late after previous RVOT surgery was reviewed. METHODS: Between August 2002 and April 2005, 31 patients (mean age 14.5 +/- 9.5 years; range: 1.2-33.1 years) underwent PVR using the Medtronic Freestyle porcine aortic root. These patients averaged two prior operations (range: 1-5) for the following diagnoses: tetralogy of Fallot +/- pulmonary atresia (n = 21); persistent truncus arteriosus (n = 5); aortic stenosis (Ross-Konno procedure) (n = 2); pulmonary atresia with intact ventricular septum; (n = 1); congenital pulmonary stenosis (n = 1); and transposition of the great arteries (n = 1). Of these patients, 29 (93.5%) underwent additional procedures at the time of pulmonary valve insertion including: branch pulmonary artery reconstruction (n = 21), atrial septal defect closure (n = 5), ascending aorta replacement (n = 4), pacemaker or defibrillator placement (n = 3), tricuspid valve repair (n = 2), ventricular septal defect closure (n = 2), and other procedures (n = 2). RESULTS: There were no early or late deaths. The mean hospital stay was 8.3 +/- 5.9 days (range: 4-25 days). One patient had a subarachnoid hemorrhage with transient left hemiparesis, and two patients had acute tubular necrosis with temporary dialysis treatment. All patients were well at a mean follow up of 13 +/- 9.3 months (range: 0.5-31 months). Echocardiography showed trivial or no pulmonary insufficiency in 30 patients (96.7%). The calculated mean peak systolic RVOT gradient by echocardiography was 23.4 +/- 7.6 mmHg. CONCLUSION: The Medtronic Freestyle bioprostheses demonstrated excellent short-term results for repeat RVOT reconstruction. This valve's hemodynamic characteristics are comparable to those of homografts, and it is an attractive alternative given the limited availability of homograft valves. A lack of availability in sizes smaller than 19 mm limits use of this valve in pediatric patients, and long-term results remain to be determined.

Use of recombinant factor VII to control bleeding in a patient supported by right ventricular assist device after heart transplantation.

A 48-year-old man undergoing orthotopic heart transplantation for ischemic cardiomyopathy developed severe right heart failure. Severe intractable bleeding complicated implantation of a right ventricular assist device. Treatment with 9.6 mg of recombinant activated factor VII stopped the bleeding, and the patient could be transferred to the intensive care unit. This is the first reported case of the successful use of recombinant activated factor VII to control bleeding with subsequent right ventricular assist device function.

Surgical management of aortopulmonary window and associated lesions.

BACKGROUND: Aortopulmonary window is a rare congenital heart defect commonly associated with other cardiac anomalies. Although single institutional experience is low, several surgical techniques have been reported. The purpose of this study is to describe our approach to the management of aortopulmonary window and its associated lesions. METHODS: Between February 1996 and November 2002, 13 patients underwent repair of aortopulmonary window. The age range went from 4 days to 5.5 months (mean 42 +/- 52 days), with 9 patients younger than 1 month old. The weight range was from 1.9 to 6.7 kg (mean 3.5 +/- 1.2 kg). Concomitant cardiac anomalies were present in 11 patients. The major additional anomalies were interruption of aortic arch in 4 patients and tracheal stenosis in 1 patient. Initial diagnoses were made using two-dimensional echocardiography only. RESULTS: There was one postoperative death. In general, patients with aortopulmonary window and additional major defects had a prolonged intensive care unit and hospital stay when compared with the other patients. Follow-up time ranged from 2 months to 6.8 years (mean of 2.5 +/- 2.2 years). There were no reoperations and no late deaths. Transcatheter balloon dilatation of the repaired aortic arch was required in 1 patient and of the right pulmonary artery in another. All other patients had good flow to both pulmonary arteries. No residual shunts were detected at the aortopulmonary window site, and pulmonary pressures were normal. CONCLUSIONS: Aortopulmonary window may be effectively diagnosed with echocardiography. Early surgical treatment (neonatal period, if possible) is safe and associated with the best long-term results, even in the presence of other cardiac anomalies. Complete separation and reconstruction of both aorta and pulmonary arteries under direct vision may prevent recurrence and distortion of adjacent structures.

Successful resuscitation of a patient with acute massive pulmonary embolism using emergent embolectomy.

Acute massive pulmonary embolism is associated with a high mortality rate. Prompt diagnosis and treatment are mandatory for a successful outcome. Although thrombolysis is effective, it is associated with a high rate of bleeding complications. This report describes the use of emergent pulmonary embolectomy as an effective and aggressive therapeutic approach to a massive saddle pulmonary embolism in a 66-year-old woman. With the application of specific surgical techniques and good interdisciplinary cooperation, pulmonary embolectomy may serve as more than a last resort for the management of this clinically unstable and dangerous condition.

Konno aortoventriculoplasty in children and adolescents: from prosthetic valves to the Ross operation.

BACKGROUND: In children with complex multilevel left ventricular outflow tract obstruction (LVOTO), the Konno aortoventriculoplasty is used to enlarge the aortic root and increase the size of the aortic valve implanted. We present our experience with the evolution of this surgical approach from prosthetic valves to the Ross operation. METHODS: Between March 1982 and July 2000, 60 patients (36 males and 24 females) had 72 Konno aortoventriculoplasties (prosthetic valve and Konno group [57 patients; redo, 12] and Ross-Konno group [15 patients]). The patients' ages ranged from 5 days to 18 years (mean, 8.2 years). The underlying anatomic diagnoses were congenital aortic stenosis and LVOTO in 51 patients, coarctation of the aorta with bicuspid aortic valve in 13, severe aortic insufficiency associated with a ventricular septal defect in 8, interrupted aortic arch in 6, mitral stenosis in 6, atrioventricular septal defect in 5, and endocarditis in 3. There was no statistical difference in age or diagnosis between the two groups. The prosthetic valve group included 42 mechanical valves, 9 homografts, and 6 xenografts. RESULTS: Follow-up ranged from 10 months to 18.5 years (mean, 9.7 years) in the prosthetic valve and Konno group compared with 5 months to 3.7 years (mean, 2.1 years) in the Ross-Konno group (p < 0.05). There were 10 early deaths and four late deaths in the prosthetic valve and Konno group as compared with one early death and two late deaths in the Ross-Konno group (p = not significant). Reoperations for LVOTO and aortic valve replacement were significantly higher in the prosthetic valve and Konno group as opposed to the Ross-Konno group (16 vs 0, p < 0.05) mainly because of the biological valve and Konno subgroup. There were no statistical differences in reexploration for bleeding, pacemaker insertion, and reoperations for indications other than LVOTO and aortic valve replacement between the two groups. CONCLUSIONS: The Konno aortoventriculoplasty is a good surgical option for complex LVOTO. Patients with a prosthetic valve and Konno carry a high rate of reoperation. Early operative results with the Ross-Konno operation seem promising.

Mitral valve replacement in children.

BACKGROUND AND AIMS OF THE STUDY: Although repair of the mitral valve in children with or without concomitant congenital heart defects has improved significantly, it is not always achievable. The study aim was to review a 20-year experience of mitral valve replacement (MVR) in children. METHODS: Since 1980, 90 patients (37 males, 53 females; mean age 8.1 years; range: 3 weeks to 18 years) have undergone a total of 102 MVR operations (12 redo-MVR, nine multiple valves, and three with common atrioventricular valve replacement). Tissue valves were used in 13 patients (14%). The etiology for valve disease was congenital in 72 patients (80%), and 34 patients had atrioventricular septal defect (AVSD). Other etiologies included rheumatic heart disease (n = 8), myxomatous disease (n = 4), endocarditis (n = 3), and Kawasaki disease, left atrial myxoma and idiopathic hypertropic subaortic stenosis (each n = 1). In total, 36 patients (40%) had a previous mitral valve repair, and 34 (38%) had concomitant repair of associated lesions. RESULTS: Hospital mortality was significantly higher in children aged < 2 years (52%, 15 of 29) compared with older children (3%, 2 of 61) (p < 0.001). Fourteen hospital deaths were associated with failed repair of complex congenital heart defects, mainly AVSD under age 2 years, followed by MVR. Mean follow up was 9.3 years (range: 7 months to 21.5 years). There were four late deaths; major events included thromboemboli (n = 6), bleeding (n = 9), endocarditis (n = 1) and cardiomyopathy with orthotopic heart transplantation (n = 7). CONCLUSION: MVR is a good surgical option for a nonrepairable mitral valve in children aged over 2 years. MVR following failed AVSD repair carries a high incidence of morbidity and mortality.

The Ross procedure as the surgical treatment of active aortic valve endocarditis.

BACKGROUND AND AIM OF THE STUDY: The authors' experience is reported of aortic valve replacement (AVR) using the pulmonary autograft in patients with active aortic valve endocarditis, including an urgent Ross procedure in infants with the acute condition. METHODS: Nine patients aged between 8 months and 38 years, with a diagnosis of aortic valve endocarditis, have undergone AVR using the Ross procedure at the authors' institution since October 1997. The diagnosis was established by clinical and echocardiographic findings. Indications for surgery were severe aortic insufficiency and congestive heart failure in all patients, with the addition of thromboembolic events (n = 3), persistent hyperpyrexia (n = 3) and vegetations (n = 5). Four infants with no history of congenital cardiac malformation underwent urgent surgery because of acute bacterial endocarditis and rapid hemodynamic deterioration. Blood cultures were positive for Streptococcus pneumoniae in three patients, and Kingella kingi and Staphylococcus aureus in one patient each. Four patients were culture-negative. All patients were treated with intravenous antibiotics for four to six weeks postoperatively. RESULTS: There were no perioperative or late deaths, and no recurrent endocarditis at the implanted valves. Echocardiographic evaluation at discharge showed trivial to mild aortic insufficiency, with no stenosis at the left ventricular outflow tract. Similar findings were found across the right ventricular outflow tract. At follow up (range: 4 months to 5.5 years), none of the patients showed progression of aortic valve insufficiency or developed stenosis; three had mild and moderate homograft stenosis (Doppler gradient 20-40 mmHg), and all children had moderate homograft insufficiency. CONCLUSION: The Ross procedure is an excellent therapeutic option for active aortic valve endocarditis in young patients, and demonstrates low morbidity and mortality. Early surgery may be indicated in patients with acute aortic valve endocarditis because of the rapidly progressive nature of this disease.

Double-valve Libman-Sacks endocarditis: an entity that demands special consideration.

BACKGROUND AND AIM OF THE STUDY: Valvular heart disease, with a propensity for the left valves, is the most important cardiac manifestation of systemic lupus erythematosus (SLE). Libman-Sacks endocarditis complicating SLE has rarely been reported to cause hemodynamically significant valvular lesions necessitating valve replacement. METHODS: This report describes a young woman with moderate aortic regurgitation and moderate to severe mitral regurgitation due to Libman-Sacks endocarditis. RESULTS: Treatment consisted of aortic and mitral valve replacement with mechanical prostheses due to intractable heart failure. The patient's recovery was uneventful. CONCLUSION: A literature survey disclosed only nine reports of double-valve replacement in patients with SLE. These findings, together with the present experience, suggest that valvular disease in SLE changes frequently with time, appears to be temporally unrelated to the other clinical features of SLE, and is associated with substantial morbidity and mortality. Corticosteroid treatment may slow the progression of valvular regurgitation. If surgery is necessary, replacement with a mechanical valve may be better than with a bioprosthesis.

Tricuspid valve papillary fibroelastoma: an unusual cause of intermittent dyspnea.

A 67-year-old woman was referred for investigation of intermittent dyspnea, which was known for years but had been worsening over the previous 3 months. Two-dimensional echocardiography revealed the presence of a mass attached to the anterior leaflet of the tricuspid valve. The patient was successfully operated on to excise the right atrial mass and preserve the tricuspid apparatus. Morphological examination of the excised tissue led to the diagnosis of papillary fibroelastoma. Surgical treatment should be considered when such a tumor is diagnosed, even in asymptomatic patients, because of the possible risk of embolization.

Transesophageal echocardiography evaluation and follow-up of left main coronary artery patch angioplasty.

OBJECTIVE: Isolated ostial stenosis of the left main coronary artery is a rare but serious condition. The treatment is surgical with two options: coronary artery bypass grafting or surgical angioplasty of the left main coronary artery. Assessing surgical results as well as follow-up were traditionally done by angiography. METHODS: We describe the use of transesophageal echocardiography (TEE) for evaluating and follow the surgical left main coronary artery (LMCA) angioplasty results in eight patients with isolated ostial left main stenosis. RESULTS: All patients were alive and free of ischemic events 8 months to 7 years post-surgery. TEE demonstrated a widely opened left main coronary artery with a good flow. CONCLUSIONS: Surgical angioplasty is an alternative option for treating ostial LMCA stenosis. TEE is an additional excellent non-invasive technique for assessing left main anatomy pre- and postoperatively, as well as being on of the quality control tools for evaluating new surgical techniques.