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.
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.
Balloon Valvuloplasty; Simultaneous Transcatheter; VSD; Pulmonary Stenosis; Balloon Catheterization
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.
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.
Eustachian valve endocarditis; transthoracic echocardiography; intravenous drug abuse; staphylococcus aureus
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.
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.
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.
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.
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.
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.
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.
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.
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.
In non-addicted patients, several states, such as permanent pacemakers, can provide the predisposing factors for tricuspid-valve endocarditis. In this report, we present a case of a 66-year-old man with pacemaker lead infection and tricuspid-native-valve endocarditis, related to Staphylococcus hominis, very rare cause of infective endocarditis that carries a high-mortality risk. Surgery was indicated for the patient due to persistent enlarging vegetation on the tricuspid valve, severe tricuspid regurgitation, septic pulmonary emboli and finally uncompensated respiratory and heart failure. Many ingenious methods have been devised to repair the tricuspid valve in patients with infective endocarditis. Valve replacement, however, is hazardous due to the possibility of prosthetic infection, and we choose to repair the native valve. The patient has now been weel for 3 years.
Dr. Lewis Dexter was an outstanding cardiovascular physiologist and clinician, a respected teacher and scientist, and, most importantly, a fine human being. During his life, he brought the cardiac catheter from the laboratory to the patient and trained several generations of cardiologists. Dexter's laboratory was the first to elucidate the pathophysiologic alterations present in many forms of congenital heart disease, including atrial septal defects, patent ductus arteriosus, tetralogy of Fallot, ventricular septal defects, and pulmonic stenosis. Subsequent work in Dexter's laboratory led to the 1st measurements of pulmonary capillary wedge pressure and to the precise calculation of stenotic valve areas from hemodynamic parameters measured during cardiac catheterization.
During a teaching exercise, Dexter demonstrated that exercise with a cardiac catheter in the heart was safe and produced clinically important data, by having a cardiac catheter inserted in himself. Over the years, many significant pathophysiologic studies that explored pulmonary embolism, valvular heart disease, right and left ventricular function, and pulmonary hypertension were published from Dexter's laboratory. But Lewis Dexter was more than a brilliant researcher. “Lew” was very close to his fellows and students, whom he considered extensions of his family. Dexter was a remarkable teacher, a compassionate physician, and a scrupulously honest investigator. Dr. Lewis Dexter had a major impact on modern medicine and was one of the great cardiologists of the 20th century.
Heart catheterization/history/instrumentation; heart defects, congenital/therapy; history of medicine, 20th cent.; pulmonary wedge pressure
Right sided endocarditis usually involves the tricuspid valve, predominantly in intravenous drug users. It is also occasionally acquired in hospital as a result of contaminated intravascular devices. Isolated infection of the pulmonary valve is rarely seen. A case of community acquired Staphylococcus aureus pulmonary valve endocarditis that caused diagnostic confusion is reported. This infection occurred in a patient with no history of intravenous drug abuse and a previously structurally normal heart.
Keywords: Staphylococcus aureus; pulmonary valve; endocarditis
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.
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). 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), 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.
calcineurin; Nfatc1; secondary heart field; semilunar valve
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.
Prosthetic valve endocarditis; Staphylococcus Capitis; Early surgery; Antibiotics
Tricuspid valve endocarditis frequently occurs in the setting of intravenous drug use. A case of tricuspid valve endocarditis in a 37-year-old woman with a history of intravenous cocaine use is described. Transthoracic echocardiography showed extension of the tricuspid valve mass through a patent foramen ovale and into the left atrium. One week after intravenous antibiotic treatment, the mass no longer traversed the patent foramen ovale, and only two smaller tricuspid valve vegetations remained. The present case demonstrates the value of performing a complete and thorough transthoracic echocardiography to visualize and evaluate both the right-and left-sided consequences of infective endocarditis in intravenous drug users. It also serves as a useful reminder to physicians caring for such patients that right-sided endocarditis can have important left-sided complications.
Echocardiography; Infective endocarditis; Patent foramen ovale
Endocarditis of the right side of the heart is otherwise uncommon in children. Pulmonary endarteritis as a complication of congenital heart disease is even rarer. Herein, we report the case of pulmonary endarteritis with a 7 mm ×5 mm vegetation, involving the main pulmonary artery in a 4-year-old male child, with cyanosis and a 1-week history of fever and rapidly-progressive hemiparesis. A full segmental echocardiography demonstrated a double inlet left ventricle with left-sided subaortic hypoplastic right ventricle (Van Praagh's A-III type – Single Ventricle). Additionally, CT scan of the brain revealed bilateral cerebral abscesses. To the best of our knowledge, the occurrence of pulmonary endarteritis and cerebral abscesses in a case of single ventricle is hitherto unreported. This article underlines the importance of heightened clinical awareness and meticulous echocardiography in cases of congenital heart disease so that relatively rare complications may not be missed.
Cerebral abscess; cyanotic heart disease; pulmonary endarteritis; single ventricle
Double orifice mitral valve is a rare congenital anomaly presenting as the division of the mitral orifice into two anatomically distinct orifices, it is most often associated with other congenital heart defects such as left-sided obstructive lesions, ventricular septal defects or aortic coarctation. We report the case of a 15 year’s old boy, admitted for arterial hypertension, auscultation revealed a rude aortic systolic murmur. Femoral pulses were weak. Owing to the suspicion of aortic coarctation, transthoracic echocardiography was performed, the aortic coarctation with dilation of the aorta proximal to the stenosis was confirmed and bicuspid aortic valve was found with good function. The mitral valve was dysmorphic, having two orifices; it was divided into 2 separate valve orifices by a fibrous bridge. No mitral or aortic regurgitation was documented by color Doppler flow imaging. The left ventricular ejection fraction was normal. There was a small peri membranous ventricular septal defect with left to right shunt. Owing to the severity of the aortic coarctation and taking into account the anatomy and characteristics of the patient, he was made a surgical correction of aortic coarctation with good outcome.
Secondary hypertension; Aortic coarctation; Bicuspid aortic valve; Double orifice mitral; Ventricular septal defect
Infective endocarditis is a serious complication of intravenous (IV) drug abuse, with a reported mortality of 5 to 10%. A 21-year-old man, who was an intravenous drug abuser, presented with fever and dyspnea. Transthoracic echocardiography showed a highly mobile, large vegetation on the anterior leaflet of the tricuspid valve. Despite antibiotic therapy for ten days, the patient remained febrile. Transesophageal echocardiography revealed severe aortic regurgitation and an echo-lucent space between the tricuspid and aortic valves. Color Doppler demonstrated a flow within the echo-lucent space and a connection between that and the left ventricle, suggesting a perivalvular abscess of the tricuspid valve opening in the left ventricle. The patient was transferred to the operating room, where he unfortunately expired.
Endocarditis; Tricuspid valve; Abscess
We describe a 42-year-old man who presented as life-threatening sepsis and septic shock with multiple septic pulmonary embolism and septic pneumonia due to pulmonary valve endocarditis. The patient had history of untreated ventricular septal defect (VSD) and complained of severe dyspnea and orthopnea. Transthoracic and transesophageal echocardiograms revealed severe pulmonary regurgitation with large, hypermobile vegetation on pulmonary valve and right ventricular outflow tract (RVOT), and a small subarterial type VSD. Emergency operation was done due to rapid deterioration of the patient, and after 6 weeks of antibiotics coverage, he was discharged.
Pulmonary valve; Endocarditis; Ventricular septal defect
Coagulase negative staphylococci are the principal cause of prosthetic valve endocarditis but are a rare cause of native valve infections. However, the incidence of native valve endocarditis is increasing. Staphylococcus capitis is a coagulase negative staphylococcus with the capacity to cause endocarditis on native heart valves. Two cases of native valve endocarditis caused by S capitis are presented; both in patients with aortic valve disease. The patients were cured with prolonged intravenous vancomycin and rifampicin and did not need surgery during the acute phase of the illness. Five of the six previously described cases of endocarditis caused by this organism occurred on native valves and responded to medical treatment alone.
Keywords: Staphylococcus capitis; endocarditis; valvar disease; coagulase negative staphylococci
Systemic infection with Aspergillus is an opportunistic disease that affects mainly immunocompromised hosts, and is associated with a high mortality rate. It typically occurs in patients with several predisposing factors, but Aspergillus endocarditis of native valves is rare and experience in diagnosis and treatment is limited. We report a case of native valve endocarditis caused by Aspergillus. A 35-yr-old male patient who underwent pericardiocentesis four months previously for pericardial effusion of unknown etiology presented with right leg pain and absence of the right femoral artery pulse. Cardiac echocardiography revealed severe mitral insufficiency with large mobile vegetations, and computed tomographic angiography showed embolic occlusion of both common iliac arteries. We performed mitral valve replacement and thromoembolectomy, and Aspergillus was identified as the vegetation. We started intravenous amphotericin B and oral itraconazole, but systemic complications developed including superior mesenteric artery aneurysm and gastrointestinal bleeding. After aggressive management, the patient was discharged 78 days post surgery on oral itraconazole. He was well at 12 months post discharge but died in a traffic accident 13 months after discharge.
Aspergillus; Endocarditis; Vascular Diseases; Surgery
The diagnosis of fungal endocarditis requires a high index of clinical suspicion. Rarely, pacemaker implantation may be a risk factor for the development of fungal endocarditis. A 71-year-old man with a history of multiple transvenous pacemaker manipulations and fever of an uncertain source is described. A diagnosis of culture-negative pacemaker endocarditis was established only after repeat transthoracic echocardiography. Amphotericin B was instituted; however, the patient developed a cerebral infarct and died. Postmortem examination demonstrated Aspergillus fumigatus within a large pacemaker lead thrombus, tricuspid and aortic valve vegetations, and septic pulmonary and renal emboli. The present report describes the clinical and pathological features of a rare case of Aspergillus fumigatus pacemaker lead endocarditis and suggests that serial echocardiograms may be effective in the early detection of pacemaker lead vegetations. The diagnostic features and therapeutic management of pacemaker lead endocarditis are reviewed.
Aspergillus fumigatus; Echocardiography; Endocarditis; Fungus; Pacemaker
A Gerbode -type defect is a ventricular septal defect communicating directly between the left ventricle and right atrium. It is usually congenital, but rarely is acquired, as a complication of endocarditis. This can be anatomically possible because the normal tricuspid valve is more apically displaced than the mitral valve. However, identification of an actual communication is often extremely difficult, so a careful and meticulous echocardiogram should be done in order to prevent echocardiographic misinterpretation of this defect as pulmonary arterial hypertension. The large systolic pressure gradient between the left ventricle and the right atrium would expectedly result in a high velocity systolic Doppler flow signal in right atrium and it can be sometimes mistakably diagnosed as tricuspid regurgitant jet simulating pulmonary arterial hypertension.
We present a rare case of young woman, with endocarditis who presented with severe pulmonary arterial hypertension. The preoperative diagnosis of left ventricle to right atrial communication (acquired Gerbode defect) was suspected initially by echocardiogram and confirmed at the time of the surgery.
A point of interest, apart from the diagnostic problem, was the explanation for its mechanism and presentation. The probability of a bacterial etiology of the defect is high in this case.
We present a case of fungal endocarditis in a 42-year-old man with a history of intravenous drug abuse who required aortic valve replacement for severe aortic insufficiency. Cultures of the resected valve grew Pseudallescheria boydii. The patient subsequently developed persistent endocarditis of the prosthetic valve with systemic embolism. At autopsy, cultures of the prosthetic valve grew Monosporium apiospermum, an anamorph of Pseudallescheria boydii. Although fungal endocarditis is not uncommon in intravenous drug abusers, endocarditis caused by Pseudallescheria boydii is rare. It has been reported in only three other instances, and under circumstances that were different from ours. (Texas Heart Institute Journal 1987; 14:321-324)
Endocarditis, fungal; Pseudallescheria; aortic valve; substance abuse