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1.  Pulmonary Valve Infective Endocarditis in an Adult Patient with Severe Congenital Pulmonary Stenosis and Ostium Secundum Atrial Septal Defect 
A hypertensive 76-year-old man with severe pulmonary valve stenosis (PVS) and recent initiation of haemodialysis was referred with fever, chills, and asthenia. One month prior, he had been admitted with similar symptoms. Transthoracic echocardiography (TTE) had shown a PVS and no valve vegetations were observed. Following discharge, he was readmitted with fever and blood cultures positive for Staphylococcus haemolyticus. A new TTE revealed two pulmonary valve vegetations and a previously undetected ostium secundum-type atrial septal defect (ASD), confirmed by transesophageal echocardiography. The clinical course was uneventful with intravenous antibiotic treatment and the patient was safely discharged. This is a case of pulmonary valve infective endocarditis (IE). The incidence of right-sided IE is on the rise due to the increased number of patients using central venous lines, pacing, haemodialysis and other intravascular devices. Pulmonary valve IE is extremely rare, especially in structurally normal hearts. The case reported here, presents a combination of predisposing factors, such as severe congenital PVS, the presence of a central venous catheter, and haemodialysis. The fact that it was an older patient with severe congenital PVS and associated with a previously undiagnosed ASD, is also an unusual feature of this case, making it even more interesting.
PMCID: PMC3014674  PMID: 21234101
2.  Eustachian valve endocarditis: a rare localization of right side endocarditis. A case report and review of the literature 
Right-sided endocarditis occurs predominantly in intravenous drug users, in patients with pacemaker or central venous lines and in patients with congenital heart disease. The vast majority of cases involve the tricuspid valve. Eustachian valve endocarditis is an uncommon disease with similar signs and symptoms of the tricuspid valve endocarditis. A series of only 16 cases of eustachian valve endocarditis are reported in the literature.
Case Presentation
We present a case of a 25-year old woman with intravenous drug abuse who had a staphylococcus aureus tricuspid valve endocarditis associated to eustachian valve endocarditis. Transthoracic echocardiography, as first line examination, showed the vegetations on tricuspid and eustachian valve.
Our case describe an unusual location of right side endocarditis in a intravenous drug abuser. In our case, in accord with other cases described in the literature, transthoracic echocardiography disclosed eustachian valve endocarditis.
Antimicrobial management is not altered by the recognition of eustachian valve endocarditis. Antibiotic treatment and duration of eustachian endocarditis depends on the isolated organism and is similar to antibiotic therapy used in native valve endocarditis.
PMCID: PMC1236943  PMID: 16171516
Eustachian valve endocarditis; transthoracic echocardiography; intravenous drug abuse; staphylococcus aureus
3.  Simultaneous Transcatheter Closure of Ventricular Septal Defect and Pulmonary Valvuloplasty 
Iranian Journal of Pediatrics  2012;22(4):555-558.
Transcatheter balloon valvuloplasty has been the accepted first line treatment for congenital pulmonic stenosis (PS) in children. Transcatheter closure of perimembranous ventricular septal defect (VSD) with Amplatzer VSD occluder is an alternative to surgical repair.
Case Presentation
A 12 year old boy presented with history of exertional dyspnea and atypical chest pain. Physical findings were suggestive of severe pulmonic stenosis. Transthoracic echocardiography, right and left ventricular angiography showed medium to large sized perimembranous VSD and severe valvar pulmonary stenosis. Transcatheter closure of VSD was done first so as to avoid large left to right shunt across VSD after balloon pulmonary valvuplasty.
We demonstrated the feasibility and success in treating combined ventricular septal defect and severe pulmonary valve stenosis with transcatheter interventional procedure in the same session.
PMCID: PMC3533162  PMID: 23429408
Balloon Valvuloplasty; Simultaneous Transcatheter; VSD; Pulmonary Stenosis; Balloon Catheterization
4.  Streptococcus Constellatus Community Acquired Pneumonia with Subsequent Isolated Pulmonic Valve Endocarditis and Abscess Formation in a Structurally Normal Heart 
Pulmonic valve infective endocarditis in isolation is a rare clinical entity. The formation of an abscess in the right ventricular outflow tract as a consequence of vegetations affecting the pulmonic valve in a structurally normal heart is extremely rare and has not been reported. We report a case of isolated pulmonic valve endocarditis complicated by a regional abscess formed within the right ventricular outflow tract caused by Streptococcus Constellatus (S. Constellatus), a member of the Streptococcus Milleri group in a young male whose risk factor was alcohol abuse and he was treated medically, a comprehensive literature review on the subject is also reported. Our case is the first reported in literature with infective endocarditis caused by S. Constellatus affecting the pulmonic valve, and the first with pulmonic valve endocarditis and perivalvular abscess formation in a structurally normal heart.
PMCID: PMC4096672  PMID: 25031801
Infective endocarditis; Pulmonary valve; Echocardiogram; Streptococcus
5.  Actinobacillus actinomycetemcomitans endocarditis in a 1.5 year old toddler 
BMJ Case Reports  2010;2010:bcr1120092462.
The authors report a 1.5-year-old girl who developed Actinobacillus actinomycetemcomitans (AA) endocarditis involving the pulmonic valve. She had a congenital cardiac abnormality, but no history of dental manipulation. The case illustrates an uncomplicated course with three unique features; the youngest reported infant with endocarditis caused by AA with vegetation on the pulmonic valve. She underwent a benign course with complete recovery.
The authors report a 1.5-year-old girl who developed AA endocarditis involving the pulmonic valve. She had a congenital cardiac abnormality, but no history of dental manipulation. The case illustrates an uncomplicated course with three unique features; the youngest reported infant with endocarditis caused by AA with vegetation on the pulmonic valve. She underwent a benign course with complete recovery.
PMCID: PMC3029670  PMID: 22767569
6.  Pulmonary valve endocarditis caused by right ventricular outflow obstruction in association with sinus of valsalva aneurysm: a case report 
Right-sided infective endocarditis is uncommon. This is primarily seen in patients with intravenous drug use, pacemaker or central venous lines, or congenital heart disease. The vast majority of cases involve the tricuspid valve. Isolated pulmonary valve endocarditis is extremely rare. We report the first case of a pulmonary valve nonbacterial thrombotic endocarditis caused by right ventricular outlflow tract (RVOT) obstruction in association with a large sinus of Valsalva aneurysm.
Case presentation
A 60-year-old man with a six-week history of fever, initially treated as pneumonia and sinusitis with levofloxacin, was admitted to the hospital with a new onset of a heart murmur. An echocardiogram showed thickening of the pulmonary valve suggestive of valve vegetation. A dilated aortic root and sinus of Valsalva aneurysm measuring at least 6.4 cm were also identified. The patient was empirically treated for infective endocarditis with vancomycin and gentamycin for 28 days. Four months later, the patient underwent resection of a large aortic root aneurysm and exploration of the pulmonary valve. During the surgery, vegetation of the pulmonary valve was confirmed. Microscopic pathological examination revealed fibrinous debris with acute inflammation and organizing fibrosis with chronic inflammation, compatible with a vegetation. Special stains were negative for bacteria and fungi.
This is the first case report of a pulmonary valve nonbacterial endocarditis caused by RVOT obstruction in association with a sinus of Valsalva aneurysm. We speculate that jets created by the RVOT obstruction and large sinus of Valsalva aneurysm hitting against endothelium of the pulmonary valve is the etiology of this rare nonbacterial thrombotic endocarditis.
PMCID: PMC2491616  PMID: 18627635
7.  Conservative surgery in multiple cusp involvement in tricuspid valve endocarditis. 
British Heart Journal  1991;66(3):244-245.
Tricuspid and mitral valve endocarditis caused by Staphylococcus epidermidis in a 57 year old previously healthy man with no history of drug abuse presented as bi-ventricular failure and multiple episodes of pulmonary emboli. He was treated for four weeks with intravenous antibiotics and had serial echocardiographic assessment of the vegetation on the tricuspid valve. This was followed by mitral valve replacement, local excision of vegetation from all the three cusps of the tricuspid valve, and autologous pericardial reconstruction of these cusps with functional assessment by perioperative transoesophageal echocardiography. Postoperative cardiac function was excellent and serial echocardiographic assessment confirmed satisfactory tricuspid valve function. This is believed to be the first recorded case in which autologous pericardial repair was used to reconstruct all the three cusps in a tricuspid valve after excision of vegetations.
PMCID: PMC1024652  PMID: 1931353
8.  New Approaches to the Diagnosis and Treatment of Infective Endocarditis 
Texas Heart Institute Journal  1989;16(4):250-257.
To assess the effect of our new, more aggressive approach to treating infective endocarditis, we retrospectively reviewed our recent experience with this disease. Between 1983 and 1989, we treated 100 patients with endocarditis, in 94 of whom the diagnosis was confirmed. Fifty-four (57%) of the 94 patients had native valve endocarditis, and the other 40 patients (43%) had prosthetic valve endocarditis. The patients' mean ages were 50 years for native valve disease and 58 years for prosthetic valve disease (p < 0.05). The male-to-female ratio was 4:1. Among the patients with confirmed endocarditis, 87 (93%) had significant underlying risk factors for endocarditis. Upon physical examination of the 94 patients, 16 (17%) were afebrile, 15 (16%) had negative blood cultures, and 26 (28%) had no cardiovascular symptoms or immunologic findings. Echocardiography was of limited value: its sensitivity was 56% for native valve endocarditis and 33% for prosthetic valve endocarditis. The ratio of affected valves was 5 aortic:4 mitral: 1 tricuspid in both forms of the disease. Viridans streptococcus was isolated in 23 (25%) of the confirmed cases, Enterococcus faecalis in 17 (18%), Staphylococcus aureus in 13 (14%), and coagulase-negative staphylococcus in 14 (15%). Gram-negative, anaerobic, and fungal organisms accounted for only 13 (14%) of the confirmed cases.
The mean duration of intravenous therapy was 29 days. Twenty (37%) of the native valve patients and 16 (40%) of the prosthetic valve patients received antibiotics on an outpatient basis. Vancomycin was used in 44 (47%) of the cases, nafcillin or ampicillin in 40 (44%), and other β-lactam agents in 9 (10%). The mean hospital stay was significantly longer for prosthetic valve endocarditis patients than for those with native valve disease (29 versus 23 days; p < 0.01). Cardiac catheterization was performed in 9 native valve patients (17%) and 6 prosthetic valve patients (15%). Valve surgery was performed in 33 native valve patients (61%) and 22 prosthetic valve patients (55%). Failure, defined as in-hospital death or recurrent endocarditis, accounted for 14 (28%) of the native valve cases (17% death and 11% relapse) and 8 (20%) of the prosthetic valve cases (10% death and 10% relapse), for an overall failure rate of 24%. The rate of failure was independent of the infecting pathogen or the type of antimicrobial therapy applied. Our experience verified that, in many patients with significant underlying risk factors, the diagnosis of endocarditis may be made on an empiric basis. (Texas Heart Institute Journal 1989;16:250-7)
PMCID: PMC326529  PMID: 15227377
Antibiotics; diagnosis; endocarditis, infective; heart valve diseases; heart valve prosthesis; infection
9.  Health Care–Associated Native Valve Endocarditis in Patients with no History of Injection Drug Use: Current Importance of Non-Nosocomial Acquisition 
Annals of internal medicine  2009;150(9):586-594.
The clinical profile and outcome of nosocomial and non-nosocomial health care–associated native valve endocarditis are not well defined.
To describe the prevalence, clinical characteristics, and outcomes of nosocomial and non-nosocomial health care–associated native valve endocarditis.
Prospective observational study.
61 hospitals in 28 countries.
Patients with definite native valve endocarditis and no history of injection drug use who were enrolled in the International Collaboration on Endocarditis–Prospective Cohort Study from June 2000 to August 2005.
Characteristics of nosocomial and non-nosocomial health care–associated native valve endocarditis cases were described and compared with those cases acquired in the community.
Health care–associated native valve endocarditis was present in 557 (34%) of 1622 patients with native valve endocarditis and no history of injection drug use (nosocomial native valve endocarditis 303 patients [54%]; non-nosocomial health care–associated native valve endocarditis 254 patients [46%]). Staphylococcus aureus was the most common cause of health care-associated native valve endocarditis (nosocomial native valve endocarditis, 47%; non-nosocomial health care–associated native valve endocarditis, 42%; p=0.3), with a notable proportion of methicillin-resistant S. aureus (nosocomial native valve endocarditis, 57%; non-nosocomial health care–associated native valve endocarditis, 41%; p=0.014). Patients with health care–associated native valve endocarditis had lower rates of cardiac surgery (41% health care–associated native valve endocarditis vs 51% community-acquired native valve endocarditis, p<0.001) and higher in-hospital mortality rates than patients with community-acquired native valve endocarditis (25% health care–associated native valve endocarditis vs. 13% community-acquired native valve endocarditis vs., p<0.001). Multivariable analysis confirmed a higher mortality associated with health care–associated native valve endocarditis (incidence risk ratio=1.20 (CI 95%, 1.03–1.61).
This study involves tertiary hospitals with cardiac surgery programs. The results may not be generalized to patient populations receiving care in other types of facility.
More than one-third of all cases of native valve endocarditis in non-drug users involve contact with health care. S. aureus is the leading cause of health care–associated native valve endocarditis. Non-nosocomial health care–associated native valve endocarditis is common, especially in the US. Patients with health care-associated and community-acquired native valve endocarditis differ in their presentation, microbiology, and outcome. By contrast, patients with nosocomial and non-nosocomial healthcare-associated endocarditis are similar.
PMCID: PMC3625649  PMID: 19414837
infective endocarditis; healthcare-associated endocarditis; nosocomial endocarditis; non-nosocomial healthcare-associated endocarditis; community-acquired endocarditis; Staphylococcal aureus endocarditis; MRSA endocarditis; Coagulase-negative staphylococcal endocarditis; Surgery; Outcome
10.  A rare localization in right-sided endocarditis diagnosed by echocardiography: A case report 
Right-sided endocarditis occurs predominantly in intravenous drug users, patients with pacemakers or central venous lines and with congenital heart diseases. The vast majority of cases involve the tricuspid valve.
Case presentation
A case of a 31-year-old woman with intravenous drug abuse who had a right-sided vegetation attached to the muscular bundle of the right ventricle is presented. Transthoracic echocardiography revealed a vegetation in the right ventricular outflow tract. Transesophageal echocardiography clearly showed that the 1.8 cm vegetation was not adherent to the pulmonary valve but attached to a muscular bundle.
Our case points to an unusual location of right-sided endocarditis in intravenous drug users. It confirms that TTE remains an easy and highly sensitive first-line examination for the diagnosis of right-sided endocarditis.
PMCID: PMC194433  PMID: 12952545
11.  Transapical transcatheter aortic valve implantation in a complex aortic surgical patient: A case involving the youngest valve-in-valve implantation with a 29 mm transcather valve 
The present article reports a case involving a 29-year-old man who developed severe cardiac failure (New York Heart Association class IV). He had a complex surgical history, beginning with the repair of an anterior sinus of Valsalva aneurysm and closure of a ventricular septal defect at eight months of age. His residual Valsalva aneurysm and mixed aortic valve disease necessitated mechanical aortic valve replacement at 14 years of age. One year later, he developed coagulase-negative staphylococcal prosthetic valve infective endocarditis, necessitating an additional replacement of his valve with a pulmonary homograft. Subsequent follow-up revealed a dilated ascending aorta (6 cm) and increased regurgitation through his homograft, with significant dilation of the left ventricle. At 20 years of age, he underwent excision of the aneurysmal ascending aorta and arch of the aorta, and the aortic valve was replaced with a 29 mm bioprosthetic valve. This proved satisfactory for nine years until he presented at Guy’s and St Thomas’ National Health Services Foundation Trust (London, United Kingdom) with severe aortic regurgitation. His logistic EuroScore was 5.9 and Parsonnet score was 17 but, due to extensive previous surgery, he was considered and accepted for transcatheter aortic valve implantation. A 29 mm Edwards Sapien valve (Edwards Lifesciences, USA) was successfully implanted using a valve-in-valve procedure. The patient remained well and symptom free at early follow-up. Technical aspects of this complex adult congenital case that, to the authors’ knowledge is the youngest case of transcatheter aortic valve implantation and the first 29 mm valve-in-valve procedure, are discussed.
PMCID: PMC3627286  PMID: 23592947
Adult congenital heart disease; Aortic regurgitation; Transcatheter valve; Transapical; Valve-in-valve; Valve replacement
12.  Maintaining tricuspid valve competence in double discordance: a challenge for the paediatric cardiologist 
Heart  1998;80(5):479-483.
Objectives—To establish the prevalence of tricuspid valve abnormalities in children with a double discordant heart (or congenitally corrected transposition of the great arteries); to study the influence of the loading conditions induced by various surgical interventions on the right and left ventricle in patients with double discordance and an abnormal tricuspid valve; and to propose a rational surgical approach.
Methods—Case notes were reviewed of 141 consecutive patients admitted in the first year of life with various types of double discordance (intact ventricular septum (group 1), ventricular septal defect (group 2), ventricular septal defect and pulmonary obstruction (group 3)). A study group of 62 patients with an abnormal tricuspid valve was selected by cross sectional echocardiography. These were followed up through palliative and open heart procedures with grading of tricuspid regurgitation.
Results—Tricuspid valve abnormalities were more common in groups 1 and 2 (60% and 56%) than in group 3 (31%). Preoperative tricuspid regurgitation was more common in group 2 (90%) than in groups 1 and 3 (38% and 36%). Ten patients in groups 1 and 2 died in the neonatal period with severe tricuspid regurgitation, associated with coarctation of the aorta in 60%. Eight patients in group 1 had no surgery and are doing well, with a competent tricuspid valve. Palliative procedures were undertaken in 28 patients: 14 had pulmonary artery banding, which resulted in a decrease in tricuspid regurgitation, 12 in group 2 by reducing the pulmonary blood flow and two in group 1 by changing the septal geometry; 14 in group 3 had an aortopulmonary shunt, which induced tricuspid regurgitation in two. Twenty patients are still alive after palliation, with stable tricuspid valve function. Repair of the tricuspid valve was unsuccessful in the three patients who underwent conventional surgery, leaving the right ventricle facing the systemic circulation. In two patients with a competent but abnormal tricuspid valve, conventional surgery induced severe tricuspid regurgitation. Of the 15 patients who underwent conventional surgery, only 10 survived (mortality 33%): eight with a tricuspid valve prosthesis and two with severe residual tricuspid regurgitation. However, tricuspid regurgitation decreased after anatomical correction (nine patients), restoring a systemic left ventricle and a subpulmonary right ventricle, even when the tricuspid valve was not repaired (five patients). Eight patients are doing well after anatomical correction (mortality 11%).
Conclusions—Tricuspid valve function in double discordance with an abnormal tricuspid valve depends on the loading conditions of both ventricles and on the septal geometry. Interventions that increase right ventricular volume or decrease left ventricular pressure are likely to induce tricuspid regurgitation, while those that decrease right ventricular volume or increase left ventricular pressure are likely to improve tricuspid valve function. Repair of the tricuspid valve always failed when the right ventricle was left in a systemic position and always succeeded when the right ventricle was placed in a subpulmonary position. These results should be taken in to account when dealing with patients with double discordance and an abnormal tricuspid valve.

 Keywords: transposition of the great arteries;  double discordance;  double switch procedures;  tricuspid valve;  paediatric cardiology;  congenitally corrected transposition
PMCID: PMC1728855  PMID: 9930048
13.  Prosthetic valve endocarditis caused by Staphylococcus capitis: report of 4 cases 
Although Staphylococcus capitis is considered to be a rare causative organism for prosthetic valve endocarditis, we report 4 such cases that were encountered at our hospital over the past 2 years. Case 1 was a 79-year-old woman who underwent aortic valve replacement with a bioprosthetic valve and presented with fever 24 days later. Transesophageal echocardiography revealed an annular abscess in the aorto-mitral continuity and mild perivalvular regurgitation. We performed emergency surgery 5 days after the diagnosis of prosthetic valve endocarditis was made. Case 2 was a 79-year-old woman presenting with fever 40 days after aortic valve replacement with a bioprosthesis. Transesophageal echocardiography showed vegetation on the valve, and she underwent urgent surgery 2 days after prosthetic valve endocarditis was diagnosed. In case 3, a 76-year-old man presented with fever 53 days after aortic valve replacement with a bioprosthesis. Vegetation on the prosthetic leaflet could be seen by transesophageal echocardiography. He underwent emergency surgery 2 days after the diagnosis of prosthetic valve endocarditis was made. Case 4 was a 68-year-old woman who collapsed at her home 106 days after aortic and mitral valve replacement with bioprosthetic valves. Percutaneous cardiopulmonary support was started immediately after massive mitral regurgitation due to prosthetic valve detachment was revealed by transesophageal echocardiography. She was transferred to our hospital by helicopter and received surgery immediately on arrival. In all cases, we re-implanted another bioprosthesis after removal of the infected valve and annular debridement. All patients recovered without severe complications after 2 months of antibiotic treatment, and none experienced re-infection during 163 to 630 days of observation. Since the time interval between diagnosis of prosthetic valve endocarditis and valve re-replacement ranged from 0 to 5 days, early surgical removal of the infected prosthesis and an appropriate course of antibiotics were attributed to good clinical outcomes in our cases.
PMCID: PMC3199242  PMID: 21978659
Prosthetic valve endocarditis; Staphylococcus Capitis; Early surgery; Antibiotics
14.  The secondary heart field is a new site of Calcineurin/Nfatc1 signaling for semilunar valve development 
Semilunar valve malformations are common human congenital heart defects. Bicuspid aortic valves occur in 2-3 percent of the population, and pulmonic valve stenosis constitutes 10% of all congenital heart disease in adults (Brickner et al., 2000)[1]. Semilunar valve defects cause valve regurgitation, stenosis, or calcification, leading to endocarditis or congestive heart failure. These complications often require prolonged medical treatment or surgical intervention. Despite the medical importance of valve disease, the regulatory pathways governing semilunar valve development are not entirely clear. In this report we investigated the spatiotemporal role of calcineurin/Nfatc1 signaling in semilunar valve development. We generated conditional knockout mice with calcineurin gene disrupted in various tissues during semilunar valve development. Our studies showed that calcineurin/Nfatc1 pathway signals in the secondary heart field (SHF) but not in the outflow tract myocardium or neural crest cells to regulate semilunar valve morphogenesis. Without SHF calcineurin/Nfatc1 signaling, the conal endocardial cushion-- the site of prospective semilunar valve formation-- first develop and then regress due to apoptosis, resulting in a striking phenotype with complete absence of the aortic and pulmonic valves, severe valve regurgitation, and perinatal lethality. This role of calcineurin/Nfatc1 signaling in the SHF is different from the requirement of calcineurin/Nfatc1 in the endocardium for semilunar valve formation (Chang et al., 2004)[2], indicating that calcineurin/Nfatc1 signals in multiple tissues to organize semilunar valve development. Also, our studies suggest distinct mechanisms of calcineurin/Nfat signaling for semilunar and atrioventricular valve morphogenesis. Therefore, we demonstrate a novel developmental mechanism in which calcineurin signals through Nfatc1 in the secondary heart field to promote semilunar valve morphogenesis, revealing a new supportive role of the secondary heart field for semilunar valve formation.
PMCID: PMC3327781  PMID: 22300732
calcineurin; Nfatc1; secondary heart field; semilunar valve
15.  Prevalence and development of additional cardiac abnormalities in 1448 patients with congenital ventricular septal defects 
Heart  2004;90(11):1321-1325.
Objective: To determine by modern echocardiographic techniques the prevalence and development of cardiac abnormalities associated with ventricular septal defect (VSD).
Methods: Consecutive patients referred to a tertiary centre for paediatric cardiology and attenders at an adult congenital heart disease clinic had details of clinical outcome prospectively recorded. Patients with VSD in association with conotruncal abnormalities, atrioventricular septal defects, and univentricular heart were not included in the study.
Results: 1448 patients with VSD were assessed between 1991 and 1998. 1127 (78%) patients had isolated defects; of these 862 (76%) were perimembranous and 265 (24%) were muscular. Of the remainder, 284 (19.6% of the total population) patients had VSD associated with one other significant cardiac abnormality: 35 (2.4%) with two and two (0.1%) with three other abnormalities. The most common associated cardiac abnormalities were infundibular pulmonary stenosis (5.8%), aortic valve prolapse (3.6%), pulmonary valvar stenosis (2.7%), osteum secundum atrial septal defect (2.2%), persistent ductus arteriosus (1.9%), and coarctation of the aorta (1.5%). In the cohort of 743 patients followed up from birth, cumulative mortality was 4% by the age of 8 years and most deaths occurred within the first year of life in infants with associated genetic abnormalities such as trisomy 13 or 18. Of the 594 patients attending the adult congenital clinic with VSD, aortic regurgitation due to aortic root prolapse developed in 45 (7.6%) patients. Most of these cases were detected before the patient was 30 years old.
Conclusions: In 22% of patients with congenital VSD there were significant associated cardiac abnormalities. Some of these abnormalities, such as aortic prolapse and regurgitation or infundibular pulmonary stenosis, may develop or progress subsequently and therefore should be sought during the initial assessment and monitored during follow up. Follow up to the age of 30 years allows the detection of most cases of aortic regurgitation. The prognosis from VSD is excellent and the risk of endocarditis in a population informed of the need for antibiotic prophylaxis is small.
PMCID: PMC1768539  PMID: 15486133
ventricular septal defect; echocardiography; aortic regurgitation: congenital heart disease
16.  Essential Role for ADAM19 in Cardiovascular Morphogenesis 
Molecular and Cellular Biology  2004;24(1):96-104.
Congenital heart disease is the most common form of human birth defects, yet much remains to be learned about its underlying causes. Here we report that mice lacking functional ADAM19 (mnemonic for a disintegrin and metalloprotease 19) exhibit severe defects in cardiac morphogenesis, including a ventricular septal defect (VSD), abnormal formation of the aortic and pulmonic valves, leading to valvular stenosis, and abnormalities of the cardiac vasculature. During mouse development, ADAM19 is highly expressed in the conotruncus and the endocardial cushion, structures that give rise to the affected heart valves and the membranous ventricular septum. ADAM19 is also highly expressed in osteoblast-like cells in the bone, yet it does not appear to be essential for bone growth and skeletal development. Most adam19−/− animals die perinatally, likely as a result of their cardiac defects. These findings raise the possibility that mutations in ADAM19 may contribute to human congenital heart valve and septal defects.
PMCID: PMC303363  PMID: 14673146
17.  Spectrum of congenital heart defects and extracardiac malformations associated with chromosomal abnormalities: results of a seven year necropsy study 
Heart  1999;82(1):34-39.
OBJECTIVE—To analyse the spectrum of congenital heart malformations, the frequency of extracardiac malformations, and the proportion of chromosome aberrations among fetuses sent for necropsy.
MATERIAL—Necropsies were performed on 815 fetuses—448 induced abortions (55%), 220 spontaneous abortions (27%), and 147 stillbirths (18%)—during a seven year period (1991-97) in the department of pathology of the Charité Medical Centre in Berlin. A congenital heart defect was identified in 129 cases (16%). For all 129 fetuses, karyotyping and an ultrasound examination had been performed.
RESULTS—Congenital heart defects were present in 22% of induced abortions (99 cases), 9% of spontaneous abortions (20 cases), and 7% of stillbirths (10 cases). The heart malformations were classified into 13 categories. A fetus with more than one defect was included only in the category of the most serious defect. The malformations in order of frequency were: ventricular septal defect (VSD) (28%), atrioventricular septal defect (AVSD) (16%), hypoplastic left heart (HLH) (16%), double outlet right ventricle (DORV) (12%), coarctation of the aorta (CoA) (6%), transposition of the great arteries (TGA) (4%), aortic valve stenosis (AoVS) (4%), tetralogy of Fallot (TOF) (3%), truncus arteriosus communis (TAC) (3%), pulmonary valve stenosis/pulmonary valve atresia (PaVS/PaVA) (3%), tricuspid atresia (TA) (3%), single ventricle (SV) (1.5%), and atrial septal defect (ASD) (0.5%). The most common congenital heart defects were VSD, AVSD, HLH, and DORV, which made up 72% of all the cases. In 11 cases the heart defect was isolated (no other cardiovascular or extracardiac malformations present), 85 cases (66%) were associated with additional cardiac malformations, 85 cases (66%) were associated with extracardiac malformations, and chromosome anomalies were detected in 43 cases (33%).
CONCLUSIONS—Fetal congenital heart malformations are common. These defects are often associated with other cardiovascular and extracardiac malformations, as well as with chromosome anomalies. Complex heart defects such as AVSD, HLH, and DORV are frequent in fetuses, as they often lead to spontaneous abortion or stillbirth or, after prenatal diagnosis, to deliberate termination of pregnancy.

Keywords: congenital heart defects; extracardiac malformations; chromosomal abnormalities; necropsy examination
PMCID: PMC1729082  PMID: 10377306
18.  Massive haemoptysis in an intravenous drug user with infective tricuspid valve endocarditis 
BMJ Case Reports  2010;2010:bcr1020092404.
Major causes of morbidity in intravenous drug users are infections. In infective endocarditis, the tricuspid valve is mainly involved. Masses can cause septic embolisms and, in rare cases, they are associated with mycotic aneurysms of pulmonary arteries that lead to severe haemorrhage.
We report the case of a young woman with a history of intravenous drug abuse and prolonged infective tricuspid valve endocarditis. Initially, echocardiography showed large masses on the anterior leaflet of the tricuspid valve and severe tricuspid regurgitation; blood cultures revealed staphylococcus and streptococcus species. Eight months after initial diagnosis, she presented with severe haemoptysis and fever. CT revealed a ruptured mycotic aneurysm of the right pulmonary artery. Lobectomy was performed immediately.
Postoperatively, the patient fully recovered. After continued antibiotic treatment, follow-up examinations showed negative echocardiographic findings and blood cultures results.
PMCID: PMC3027946  PMID: 22767369
19.  Isolated pulmonary valve infective endocarditis in a middle aged man caused by Candida albicans: a case report 
BMC Infectious Diseases  2014;14(1):557.
Pulmonary valve endocarditis without the involvement of other valves represents 1.5-2% of all cases of infective endocarditis. Isolated pulmonary valve endocarditis caused by Candida is extremely rare with only one reported case in the literature and none reported in the United States. Guidelines for management of Candida endocarditis recommend a combination of medical and surgical therapy.
Case presentation
A 61-year-old homeless male presented with fever, cough and shortness of breath. He was urgently intubated for hypoxia. He was initially diagnosed with pneumonia but did not improve with empiric antibacterial therapy. Candida species were isolated from bronchoalveolar lavage fluid and the patient eventually developed persistent C. albicans bloodstream infection. On further workup he was found to have infective endocarditis with a large vegetation across the pulmonary valve. No other valves were involved. He was treated with intravenous antifungal therapy for eight weeks. Valvular surgery was not performed. Follow up echocardiography after completion of therapy did not show any vegetations and the patient clinically improved.
This is the second reported case of isolated pulmonary valve endocarditis caused by Candida and the first to be successfully managed with antifungal therapy alone. Pulmonary valve endocarditis should be considered in cases of pneumonia with Candida and persistent fungemia. While surgery should be considered in all cases of Candida endocarditis, cure may be achieved with antifungal therapy alone.
PMCID: PMC4221716  PMID: 25358458
Pulmonary valve; Candida albicans; Fungal endocarditis
20.  Staphylococcus lugdunensis Endocarditis Complicated by Embolism in an 18-Year-Old Woman with Mitral Valve Prolapse 
Staphylococcus lugdunensis is a coagulase-negative staphylococcus (CNS). It is a major cause of prosthetic valve endocarditis; mitral valve prolapse (MVP) has emerged as a prominent predisposing structural cardiac abnormality. We describe a case of Staphylococcus lugdunensis endocarditis in an 18-year-old woman with preexisting mitral valve prolapse complaining of fever, a one-month history of continuous-remittent fever (Tmax 38.6°C). The transthoracic echocardiogram revealed large vegetation on the anterior mitral valve leaflet flopping from the atrial side to the ventricular side. Five sets of blood cultures were positive for coagulase-negative staphylococci. During hospitalization, after two weeks of antibiotic therapy, the patient complained of sudden pain in her right leg associated with numbness. Lower limb arterial Doppler ultrasound showed an arterial thrombosis of right common iliac artery. Transfemoral iliac embolectomy was promptly performed and on septic embolus S. lugdunensis with the same antibiotic sensitivity and the same MIC values was again isolated. Our patient underwent cardiac surgery: triangular resection of the A2 with removal of infected tissue including vegetation. Our case is an example of infective endocarditis by S. lugdunensis on native mitral valve in a young woman of 18 with anamnesis valve prolapse.
PMCID: PMC3570945  PMID: 23424690
21.  Clinical and microbiological profiles of infective endocarditis in a tertiary hospital in Aseer region, Saudi Arabia 
We aimed to evaluate demographic data, underlying cardiac abnormalities, clinical profile, microbiological features, treatments and complications of infective endocarditis (IE) in a tertiary hospital in Aseer region, Saudi Arabia.
A retrospective study of all cases with the diagnosis of definite endocarditis according to modified Duke Criteria admitted to ACH between May 2002 and April 2007. Data were reviewed on demographic and clinical data, underlying cardiac disease, microbiological findings, treatments and complications of IE.
The study included 44 patients (28 males and 16 females; mean age 31.1 ± 16 years; range 13–65 years). Infective endocarditis developed on a native valve in 31 (70.5%), a mechanical prosthetic valve in 10 (22.7%), mitral valve prolapse in 2 (4.5%) and ventricular septal defect in 1 (2.3%). Rheumatic heart disease in 31 cases (70.5%) was the most common preexisting valvular abnormality in native valve endocarditis. The mitral valve was the most commonly affected valve 28 (63.6%). Fever occurred in 40 (90.9%) of the cases. Electrocardiography was abnormal in 34 cases (77.3%). Trans-thoracic and/or trans-esophageal echocardiography showed a vegetation in 22 (50%). Staphylococci in 10 cases (22.7%) and Streptococci in 8 cases (18%) were the most common causative agents and cultures were negative in 20 cases (45.5%). Twenty-two patients (50%) underwent surgical treatment. Congestive heart failure occurred in 16 (36.4%) cases, atrial fibrillation in 6 (13.6%) cases, and cerebrovascular accidents in 4 (9%) cases.
Our data reflects the clinical and microbiological profiles of IE in a tertiary hospital in Aseer region, Saudi Arabia.
PMCID: PMC3727542  PMID: 23960650
Endocarditis; Echocardiography; Microbiologic; Culture negative; Valvular heart disease; Prosthetic valve; Saudi Arabia
22.  Pulmonary Stenosis as a Predisposing Factor for Infective Endocarditis in a Patient with Noonan Syndrome 
Texas Heart Institute Journal  2010;37(1):99-101.
Noonan syndrome is an autosomal dominant dysmorphic syndrome. Pulmonary stenosis is the most common cardiac anomaly in Noonan patients, with an incidence of 60%. A 9-year-old girl was referred to our institution with pericardial effusion. Transthoracic echocardiography indeed confirmed massive pericardial effusion and revealed, further, valvular and arterial pulmonary vegetations that accompanied a dysplastic tricuspid pulmonary valve. We decided to perform emergency pericardial tube drainage and to continue the anti-biotic regimen for 2 more weeks before undertaking open-heart surgery. After 2 weeks, the patient underwent an operation wherein the valvular vegetations were excised and a pulmonary valve commissurotomy was performed, yielding a competent pulmonary valve with 3 distinct but moderately dysplastic cusps. In addition to the pulmonary valve, the main, left, and right pulmonary arteries were filled with mobile vegetations, which were removed during the procedure.
In this patient, a dysplastic and stenotic pulmonary valve may have contributed to the progression of endocarditis and to the growth of vegetations that occupied the pulmonary arteries. In conclusion, we hypothesize that although pulmonary stenosis is not considered a common predisposing factor for infective endocarditis, it can contribute to the progression of infective endocarditis in Noonan patients.
PMCID: PMC2829787  PMID: 20200638
Endocarditis; heart defects, congenital; pulmonary artery/surgery; pulmonary valve stenosis/complications; Noonan syndrome/therapy
23.  Patent arterial duct 
Patent arterial duct (PAD) is a congenital heart abnormality defined as persistent patency in term infants older than three months. Isolated PAD is found in around 1 in 2000 full term infants. A higher prevalence is found in preterm infants, especially those with low birth weight. The female to male ratio is 2:1. Most patients are asymptomatic when the duct is small. With a moderate-to-large duct, a characteristic continuous heart murmur (loudest in the left upper chest or infraclavicular area) is typical. The precordium may be hyperactive and peripheral pulses are bounding with a wide pulse pressure. Tachycardia, exertional dyspnoea, laboured breathing, fatigue or poor growth are common. Large shunts may lead to failure to thrive, recurrent infection of the upper respiratory tract and congestive heart failure. In the majority of cases of PAD there is no identifiable cause. Persistence of the duct is associated with chromosomal aberrations, asphyxia at birth, birth at high altitude and congenital rubella. Occasional cases are associated with specific genetic defects (trisomy 21 and 18, and the Rubinstein-Taybi and CHARGE syndromes). Familial occurrence of PAD is uncommon and the usual mechanism of inheritance is considered to be polygenic with a recurrence risk of 3%. Rare families with isolated PAD have been described in which the mode of inheritance appears to be dominant or recessive. Familial incidence of PAD has also been linked to Char syndrome, familial thoracic aortic aneurysm/dissection associated with patent arterial duct, and familial patent arterial duct and bicuspid aortic valve associated with hand abnormalities. Diagnosis is based on clinical examination and confirmed with transthoracic echocardiography. Assessment of ductal blood flow can be made using colour flow mapping and pulsed wave Doppler. Antenatal diagnosis is not possible, as PAD is a normal structure during antenatal life. Conditions with signs and symptoms of pulmonary overcirculation secondary to a left-to-right shunt must be excluded. Coronary, systemic and pulmonary arteriovenous fistula, peripheral pulmonary stenosis and ventricular septal defect with aortic regurgitation and collateral vessels must be differentiated from PAD on echocardiogram. In preterm infants with symptomatic heart failure secondary to PAD, treatment may be achieved by surgical ligation or with medical therapy blocking prostaglandin synthesis (indomethacin or ibuprofen). Transcatheter closure of the duct is usually indicated in older children. PAD in preterm and low birth weight infants is associated with significant co-morbidity and mortality due to haemodynamic instability. Asymptomatic patients with a small duct have a normal vital prognosis but have a lifetime risk of endocarditis. Patients with moderate-to-large ducts with significant haemodynamic alterations may develop irreversible changes to pulmonary vascularity and pulmonary hypertension.
PMCID: PMC2716300  PMID: 19591690
24.  Combined computed tomography and fluorodeoxyglucose positron emission tomography in the diagnosis of prosthetic valve endocarditis: a case series 
BMC Research Notes  2014;7:32.
The diagnosis of prosthetic valve endocarditis is challenging. The gold standard for prosthetic valve endocarditis diagnosis is trans-esophageal echocardiography. However, trans-esophageal echocardiography may result in negative findings or yield images difficult to differentiate from thrombus in patients with prosthetic valve endocarditis. Combined computed tomography and fluorodeoxyglucose positron emission tomography is a potentially promising diagnostic tool for several infectious conditions and it has also been employed in patients with prosthetic valve endocarditis but data are still scant.
Case presentations
We reviewed the charts of 6 patients with prosthetic aortic valves evaluated for suspicion of prosthetic valve endocarditis, at two different hospital, over a 3-year period. We found 3 patients with early-onset PVE cases and blood cultures yielding Pseudomonas aeruginosa, Staphylococcus epidermidis and Staphylococcus lugdunensis, respectively; and 3 late-onset cases in the remaining 3 patients with isolation in the blood of Streptococcus bovis, Candida albicans and P. aeruginosa, respectively. Initial trans-esophageal echocardiography was negative in all the patients, while fluorodeoxyglucose positron emission tomography showed images suspicious for prosthetic valve endocarditis. In 4 out of 6 patients valve replacement was done with histology confirming the prosthetic valve endocarditis diagnosis. After an adequate course of antibiotic therapy fluorodeoxyglucose positron emission tomography showed resolution of prosthetic valve endocarditis in all the patients.
Our experience confirms the potential role of fluoroseoxyglucose positron emission tomography in the diagnosis and follow-up of prosthetic valve endocarditis.
PMCID: PMC3899623  PMID: 24418206
FDG-PET; Prosthetic valve endocarditis; Diagnosis; Duke’s criteria
25.  Triple valve endocarditis by mycobacterium tuberculosis. A case report 
BMC Infectious Diseases  2012;12:231.
Granulomas caused by Mycobacterium Tuberculosis have been observed at autopsy in the heart, pre-dominantly in the myocardium and endocardium, but rarely involving the coronary vessels and valvular structures. Mycobacterium tuberculosis valvular endocarditis is extremely rare, with most reports coming from autopsy series.
Case presentation
We report the case of a 17 year old immunocompetent girl who presented with history of fever, malaise, foot gangrene and a left sided hemiparesis. On investigation she was found to have infective endocarditis involving the aortic, mitral and tricuspid valves. She had developed a right middle cerebral artery stroke. She underwent dual valve replacement and tricuspid repair. The vegetations showed granulomatous inflammation but blood cultures and other biological specimen cultures were negative for any organisms. She was started on antituberculous treatment and anticoagulation.
This is the first reported case of triple valve endocarditis by Mycobacterium Tuberculosis in an immunocompetent host. Especially important is the fact that the right heart is involved which has been historically described in the setting of intravenous drug abuse.
This implies that Tuberculosis should be considered in cases of culture negative endocarditis in endemic areas like Pakistan even in immunocompetent hosts.
PMCID: PMC3511257  PMID: 23013220
Immunocompetent; Endocarditis; Mycobacterium tuberculosis

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