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PubMed2005

Amelioration of hypertrophic cardiomyopathy using nonsurgical septal ablation in a cirrhotic patient prior to liver transplantation.

A 53-year-old male with hepatitis C cirrhosis, who had been refused liver transplantation because of hypertrophic cardiomyopathy (HC), underwent nonsurgical septal ablation using alcohol with resolution of his ventricular outflow obstruction. This patient was able to subsequently undergo a successful deceased donor liver transplantation. This is the first reported case of alcohol induced septal ablation being performed in a cirrhotic patient with HC. Such nonsurgical procedures may be attractive in cirrhotic patients who are refused access to liver transplantation because of high surgical risk.

Cardiomyopathy, Hypertrophic↗
PubMed2005

Gender-related differences in the clinical presentation and outcome of hypertrophic cardiomyopathy.

OBJECTIVES: The goal of this study was to assess gender-related differences in a multicenter population with hypertrophic cardiomyopathy (HCM). BACKGROUND: Little is known regarding the impact of gender on the heterogeneous clinical profile and clinical course of HCM. METHODS: We studied 969 consecutive HCM patients from Italy and the U.S. followed over 6.2 +/- 6.1 years. RESULTS: Male patients had a 3:2 predominance (59%), similar in Italy and the U.S. (p = 0.24). At initial evaluation, female patients were older and more symptomatic than male patients (47 +/- 23 years vs. 38 +/- 18 years; p < 0.001; mean New York Heart Association [NYHA] functional class 1.8 +/- 0.8 vs. 1.4 +/- 0.6; p < 0.001), and more frequently showed left ventricular outflow obstruction (37% vs. 23%; p < 0.001). Moreover, female patients were less often diagnosed fortuitously by routine medical examination (23% vs. 41% in male patients, p < 0.001). Female gender was independently associated with the risk of symptom progression to NYHA functional classes III/IV or death from heart failure or stroke compared with male gender (independent relative hazard 1.5; p < 0.001), particularly patients > or =50 years of age and with resting outflow obstruction (p < 0.005). Hypertrophic cardiomyopathy-related mortality and risk of sudden death were similar in men and women. CONCLUSIONS: Women with HCM were under-represented, older, and more symptomatic than men, and showed higher risk of progression to advanced heart failure or death, often associated with outflow obstruction. These gender-specific differences suggest that social, endocrine, or genetic factors may affect the diagnosis and clinical course of HCM. A heightened suspicion for HCM in women may allow for timely implementation of treatment strategies, including relief of obstruction and prevention of sudden death or stroke.

Adult↗
PubMed1990

Right ventricular outflow construction for complex congenital cardiac malformations without cardiopulmonary bypass: management and outcome of 44 patients.

A review of surgical and anesthetic techniques used in 44 consecutive patients to establish continuity between the right ventricle and pulmonary circulation, without extracorporeal circulation, as a first-stage repair in a variety of selected complex congenital cardiac lesions with right ventricular outflow obstruction, is presented. The overall operative mortality rate was 9% (four deaths), but no deaths occurred in the last 24 patients. Eleven patients (27.5%) have subsequently undergone complete repair and one patient (2.5%) underwent a Fontan repair. The advantages, anesthetic concerns, complications, and outcome are discussed.

Abnormalities, Multiple↗
PubMed1987

[Left ventricular function at rest and during exercise and the effects of propranolol in patients with hypertrophic cardiomyopathy].

To determine left ventricular function during the stress of exercise and to evaluate the effects of propranolol on it in patients with hypertrophic cardiomyopathy (HCM), the ECG-multigated blood pool cardiac scintigraphy was performed in 12 without (HNCM) and 10 with (HOCM) left ventricular outflow obstruction at rest and during bicycle ergometer loading. The results were compared to those in 13 normal subjects. 1. Control measurements: Resting and exercise heart rates and blood pressures did not significantly differ between patients with hypertrophic cardiomyopathy and normal subjects. Ejection fraction (EF) was normal or higher in HCM than in normal subjects at rest, but during exercise it failed to increase normally. The increment in cardiac output was half of the normal value. Responses of count-derived end-diastolic volume (EDV) and stroke volume (SV) were different between HOCM and HNCM. They were unchanged during exercise in HNCM and declined in HOCM. End-systolic volume (ESV) was unchanged in HNCM and slightly decreased in HOCM. Responses of the peak ejection rate (PER) and time to the peak ejection rate (TPE) were not significantly different from normal subjects. Left ventricular (LV) ejection time (ET) and TPE/ET were not different from normal subjects. The peak filling rate (PFR) was significantly higher than normal in HOCM at rest and during exercise. The time to peak filling rate was prolonged at rest in HOCM, but not during exercise. 2. Effects of propranolol: Heart rates significantly decreased especially after intravenous propranolol administration. Cardiac output (CO) and SV were unchanged. EDV and ESV increased significantly. The PER was unchanged. The PFR decreased, but not significantly. TPE, time to the PFR (TPF) and ET were prolonged. The ratio of TPE/ET decreased initially, but abruptly increased later. In conclusion, in the control state of HCM patients, the responses of LVEF, EDV and ESV to exercise were abnormal and CO remained half of the normal. There was no difference between patients with and without obstruction. The effect of propranolol on left ventricular function was more obvious in patients with obstruction than without obstruction, especially in ESV, EDV, PFR and TPF. Propranolol did not improve LV systolic and diastolic functions, but the double product decreased depending on the decrease of heart rate maintaining CO by Frank-Starling mechanism due to increased EDV, which may play a part in improving subjective complaints.

Administration, Oral↗
PubMed1998

Morphological characterization of ventricular septal defect with posterior deviation of the outlet septum.

Ventricular septal defect (VSD) with posterior deviation of the outlet septum is frequently associated with left ventricular outflow obstruction and aortic arch anomalies. In this study, 17 patients with large VSD and posterior deviation of the outlet septum were evaluated. The VSD was further classified into 'high' and 'low' types according to the extension of the VSD; the high-type VSD (8 patients) extended mainly to the outlet or infundibular portion and the low-type VSD (9 patients) extended mainly to the trabecular or inlet portion. Related morphological features, such as outlet septum hypoplasia, muscle bundle between aortic-mitral valve junction, pulmonary artery overriding and aortic arch anomalies, were found more frequently in patients with high-type VSD, while tricuspid valve straddling and outlet septum hypertrophy were found more often in patients with low-type VSD. This classification for VSD with posterior deviation of the outlet septum helps to predict the morphological features of left ventricular outflow tract and aortic arch anomalies.

Echocardiography↗
PubMed1988

Arterial repair for transposition of the great arteries and large ventricular septal defect in early infancy.

Arterial repair for transposition of the great arteries and large ventricular septal defect (VSD) may be superior to atrial correction, but the risk of either approach in early infancy has been high. The results of early correction were therefore assessed in 12 children with transposition of the great arteries and a large VSD who underwent arterial repair. Patients ranged in age from 3 to 90 days (median age, 19 days) and in weight from 2.5 to 3.7 kg. The VSD was infundibular in eight, inlet in three, and muscular in one. Coronary artery anomalies were present in five patients, including one patient with a single left coronary artery. There was one early death (8%) in the only patient with a pulmonary artery band. There were no late deaths. The 11 survivors have been followed up from 2 to 59 months (mean follow-up period, 20 months) and remain free of cardiac symptoms. Catheterization (n = 5) and Doppler echocardiography in all patients show no significant left ventricular outflow obstruction, aortic insufficiency, or residual VSD. Catheterization documented normal pulmonary artery pressure and unobstructed coronary arteries. Only one patient had significant pulmonary stenosis and underwent successful reoperation. These data indicate that arterial repair and VSD closure can be successfully performed in early infancy with low mortality and morbidity. Repair in this age group is advocated before changes of pulmonary vascular disease occur.

Angiography↗
PubMed1991

Evolution of the electrocardiographic changes in patients with hypertrophic cardiomyopathy.

In 75 patients with hypertrophic cardiomopathy (HCM) followed for a mean period of 5.5 years (range 2-20 years), evolution of the electrocardiographic (EKG) changes was assessed. Progression of EKG abnormalities occurred in 35 patients (47%). It was manifested by an increase in precordial QRS voltage in 33 patients, development of new P-wave mitral in 11 patients, and development or disappearance of pathologic Q waves in 14 patients. With follow-up times greater than 5 years, 5-8 years, and greater than 8 years, EKG progression was present in 27, 41, and 80% of patients, respectively. Age less than 30 years at the beginning of study and left ventricular outflow obstruction predisposed to EKG progression within 5-8 years. Patients with progressive EKG changes were more prone to experience clinical deterioration than those without EKG progression (63 vs. 15%, p less than 0.001). With chronic verapamil administration, progression of EKG abnormalities occurred insignificantly less often than with propranolol treatment (35 vs. 64%, p = 0.20). It is concluded that with long-term follow-up, HCM tends to progress in a significant proportion of adult patients.

Adolescent↗
PubMed1981

[Organic subaortic stenosis. Surgical indications, surgical technic, late results].

Between 1966 and 1979 60 patients underwent resection of organic subaortic stenosis at the National Heart Hospital. There was a male:female ratio of 40:20 and an age range of 4 to 56 years (mean 18 years). 22 patients (36.7%) had a single or multiple additional cardiac abnormalities including left ventricular outflow tract obstruction (12 cases), right ventricular outflow tract obstruction (3 cases), ventricular septal defect (6 cases), patent ductus arteriosus (5 cases), aortic coarctation (1 case), congenital mitral stenosis (1 case) and mitral atresia associated with a single ventricle (1 case). Isolated resection of the organic sub-aortic with myotomy and wedge resection was performed in 47 patients and Cooley technique in 2 patients. Total aortic root replacement with a homograft valve was necessary in 4 patients and the 22 patients additional cardiac abnormalities received concomitant surgery. There was one early death (2.6%) amongst the 38 patients with isolated organic sub-aortic stenosis and no late deaths. There were 3 early deaths (13.7%) amongst the 22 patients with additional cardiac abnormalities and one late sudden death after 14 months. There were 7 non-fatal complications: a myocardial infarction, AV block and pacemaker insertion; three mild aortic regurgitations; one mild mitral regurgitation; an arrhythmia due to Wolf Parkinson White syndrome; and an aortic and mitral valve replacement after 9 years. 43 other patients have been followed for 1 to 14 years and are symptom free. Left bundle branch block is present in two of them. Resection of organic sub-aortic stenosis with myotomy and wedge resection is a safe technique and offers an excellent prognosis for this group of congenitally compromised patients.

Adolescent↗
PubMed1992

[Accessory tricuspid valve--a report of two cases].

Accessory tricuspid valve is a rare cardiac anomaly. We describe two cases with accessory tricuspid valve. Case 1: A 7-year-old boy underwent repair of tetralogy of Fallot. Under deep hypothermic cardiopulmonary bypass, right ventricle was opened. An accessory tricuspid leaflet was noted to the right side of the VSD attaching to the infundibular septum from medial papillary muscle as a chordal origin. After excision of the accessory tricuspid leaflet, the VSD was closed with a Dacron patch followed by relieve of the right ventricular outflow obstruction and pulmonary valvotomy. Case 2: A 8-year-old boy had Rastelli operation to correct transposition of the great arteries. An accessory leaflet originated from the medial papillary muscle and was floating like a parachute in the way between VSD and aorta. There was no additional apparatus in the pathway between the VSD and aorta after excision of the accessory tricuspid leaflet. Intraventricular tunnel was created with a Dacron patch. Extracardiac conduit between the right ventricle and the distal pulmonary artery was constructed using a valved pericardial roll.

Child↗
PubMed1994

[Dynamic obstruction of the left ventricular outflow tract: surgical problems].

Dynamic subaortic obstruction caused by septal hypertrophy may worsen the hemodynamics in some patients after heart surgery. Resection of the hypertrophied septum allows normal left ventricular function. We describe two patients with subaortic stenosis and a complicated postoperative course following heart surgery and additional resection of the subaortic muscular stenosis. One patient was reoperated because of residual obstruction of the left ventricular outflow tract; the postoperative course was uneventful thereafter. The second patient died after the primary surgery because of low output syndrome and sepsis. The postoperative management of this group of patients is difficult, demanding frequent assessment of the patient's hemodynamic condition and echocardiographic surveillance.

Aged↗
PubMed1990

Pulmonary arteries: MR imaging in patients with congenital obstruction of the right ventricular outflow tract.

Thirty-five patients with congenital obstruction of the right ventricular outflow tract underwent magnetic resonance (MR) imaging for evaluation of the pulmonary arteries. All patients underwent cardiac catheterization, and 33 underwent two-dimensional Doppler echocardiography. Results of the three imaging studies were compared. With regard to the presence of a pulmonary confluence, results of MR imaging and angiography were in agreement in 31 of the 35 patients (89%), results of echocardiography and MR imaging were in agreement in 27 of 33 patients (82%), and results of echocardiography and angiography were in agreement in 27 of 33 patients (82%). There was good correlation of measurements of main pulmonary artery size among all three methods and of right and left pulmonary artery size obtained at MR imaging and angiography. Echocardiographic measurements of right and left pulmonary artery size correlated poorly with those obtained at MR imaging and angiography. Results indicated that MR imaging is useful in evaluating the pulmonary arteries in patients with congenital heart disease.

Angiography↗
PubMed2004

[Hemorrhagic nonspecific pericardial effusion as an initial symptom of angiosarcoma of the right heart].

HISTORY: A 45-year old woman presented at our hospital with intermittent fever in the last three weeks and progressive exertional dyspnea. In addition she suffered from physical asthenia. Seven months ago the patient was treated for symptomatic pericardial effusion in our clinic. INVESTIGATIONS: Physical examination revealed a pale skin color, epigastric pain on palpation and intense exertional dyspnea. Laboratory tests indicated microcytic anemia and high levels of LDH and CRP. The diagnosis of a large tumor of the right atrium and ventricle was based on the transthoracic echocardiogram and thoracic CT scan. The extent of the right atrial and ventricular mass of 9 x 8 cm was detected by trans-esophageal echocardiography. The thoracic and abdominal CT scan showed multiple nodular infiltrates in the basal lung fields and a cystic tumorous mass in the liver. DIAGNOSIS: The tumor developed in the short time of 7 months. Half a year ago, the transthoracic echocardiographic exam showed pericardial effusion with normal cardiac size, normal left ventricular function and no evidence of right ventricular outflow obstruction. No cardiac masses were observed. At that time pericardiocentesis was performed, but the pericardial fluid was cytologically negative for tumor cells. The diagnosis of angiosarcoma was made after surgical excision of the tumor and histological examination. There was only the possibility of a palliative therapy, because of the existence of pulmonary and liver metastases. CONCLUSIONS: Primary malignant cardiac tumors are rare and their prognosis is very poor. The heart angiosarcoma is often disseminated into the lungs and the liver at the time of clinical presentation. This case indicates that hemorrhagic nonspecific pericardial effusion, negative for tumor cells, can appear any time before a heart angiosarcoma is detectable by echocardiography.

Disease Progression↗
PubMed1998

[Reoperation after corrective surgery for tetralogy of Fallot].

Thirty-six patients (7.6%) underwent reoperation after total correction of tetralogy of Fallot in our institution from 1955 to 1997. Eight patients underwent a second reoperation in the same follow-up period. The indications for surgical repair were recurrent or residual lesions alone (Qp/Qs > or = 2.0, right ventricule-pulmonary artery pressure gradient > or = 50 mmHg, tricuspid regurgitation: (TR) > or = grade 3, pulmonary regurgitation (PR) > or grade 3), or in combination with other lesions. The reoperation consisted of closure of a residual VSD in 12 patients (33.3%), relief of right ventricular outflow obstruction in 12 (33.3%), tricuspid valve repair or replacement for TR in 11 (30.6%), and pulmonary valve repair or replacement for PR in 10 (27.8%). The indications for second reoperation consisted of mainly residual or recurrent TR or infections endocarditis. There was one operative death (2.8%) in the reoperation group and one death (12.5%) in the second reoperation group. Five patients (14.7%) died during a follow-up period of 1-25 years (mean 11.6 years). Twenty-none patients (80.6 %) survived surgery, and 25 (86.2%) are now in NYHA class I and 4 in class II. Reoperation and second reoperation are associated with a low mortality rate and good long-term results in our surgical experience.

Adolescent↗
PubMed1992

Chest pain secondary to membranous subaortic stenosis in a young woman.

Left ventricular outflow obstruction can be divided into three distinct categories: valvular aortic stenosis, the most common form; supravalvular aortic stenosis, which is often seen in early childhood; and subvalvular stenosis, which can be further subdivided into muscular, tunnel, and fibromembranous subtypes. All may be found in a patient seen with symptoms of angina, syncope, or heart failure as a precursor to sudden death. Prompt clinical recognition is essential as is a high degree of suspicion when these signs are associated with a systolic ejection murmur on clinical examination. Echocardiography and a meticulous Doppler examination are very useful in the diagnosis of these disorders as well as in further distinguishing among the different subtypes. The authors describe the case of a 40-year-old woman with chest pain refractory to medical therapy and a long systolic ejection murmur.

Adult↗
PubMed2000

[Long-term effect of polystan monocusp ventricular outflow patch after right ventricular outflow tract reconstruction].

Forty-eight patients who underwent right ventricular outflow tract reconstruction with Monocusp Ventricular Outflow Patch (MVOP) fifty-five times and survived surgery, were reviewed in this study. Mean age at surgery was 6.4 years-old and mean follow-up interval was 75.2 months. There was no late death, however reoperation was performed 7 times. Freedom from reoperation rate was 97.2% and 80.7% after 5 and 10 years after surgery, respectively. The main cause for reoperation were right ventricular outflow obstruction RVOTO (5 cases). All of the RVOTO occurred at the distal end of the anastomosis. However, there was no RVOTO in patients who underwent RVOTR with MVOP during the past ten years. So, we considered the cause of RVOTO a technical problem. Pulmonary regurgitation was one to two degree early after surgery, and had worsened by almost two or three degrees more than 5 years after surgery. Moreover, five of six patients who underwent cardiac catheterization more than 10 years after surgery had three degrees of pulmonary regurgitation as well as a large CTR. In conclusion, according to long-term results, especially more than 10 years post operatively, pulmonary regurgitation was the most important problem.

Adolescent↗
PubMed1981

Mitral annulus calcification, systolic anterior motion of the anterior mitral leaflet and outflow obstruction in two patients without hypertrophic cardiomyopathy. An echocardiographic report.

Systolic anterior motion of the anterior mitral leaflet (SAM) and concomitant left ventricular outflow obstruction (LVOFO) are commonly seen in hypertrophic cardiomyopathy. However, SAM has also been described together with extensive anteroseptal wall infarction, pericardial exudation and in hypovolemic situations. This report presents two patients examined with M-mode echocardiography which demonstrates that SAM with LVOFO can also occur in association with mitral annulus calcification (MAC). A possible mechanism behind this entity would firstly be the anterior displacement of the mitral ring commonly seen in MAC. Secondly, extension of calcifications to the posterior wall will furthermore impair the LV contraction leading to loosely stretched chordae tendineae during systole and hence a motion of the anterior mitral valve along the blood stream (SAM) leading to LVOFO.

Adult↗
PubMed2006

Transesophageal echocardiography in the intensive care unit.

Transesophageal echocardiography provides unique diagnostic capabilities, allowing for a very precise look at the structure and hemodynamics of the human heart. It is minimally invasive and portable, and quickly diagnoses sudden hemodynamic changes in intensive care patients. It provides invaluable and precise information about myocardial dysfunction and intracardiac volume status. It can diagnose dynamic left ventricular outflow obstruction, infrequent but serious complication of aortic valve replacement, septal myectomy, or mitral valve repair. Transesophageal echocardiography examination can exclude cardiac tamponade and intracardiac source of embolization, and it offers the ability to visualize native or prosthetic valves and assesses their function in the postoperative period. It is helpful in diagnosing endocarditis and the presence of intracardiac masses. In the diagnosis of blunt chest trauma, transesophageal echocardiography offers a fast and safe look at ascending and descending aorta and pericardial effusion, facilitating future decisions about patient management. In patients with postoperative hypoxia, it can exclude intracardiac shunt. Finally, in heart transplants or in managing patients with mechanical heart assist devices, transesophageal echocardiography is an invaluable tool in assessing progress of treatment and complications arising from the procedures. With the introduction of multiplane transesophageal echocardiography probes, technology, and experienced personnel, transesophageal echocardiography becomes the extension of the physical examination in the intensive care unit. This example is one of only a few whereby technology brings the physician closer to the patient.

Aortic Dissection↗
PubMed1998

Late results and reintervention after aortic valvotomy for critical aortic stenosis in neonates and infants.

BACKGROUND: Many centers have adopted balloon valvuloplasty for treatment of infants with critical aortic stenosis because of historically poor early results and a lack of long-term results with surgical valvotomy. We evaluated our results with open aortic valvotomy over the past decade, specifically examining factors influencing survival and reintervention in the current era. METHODS: From 1986 to 1996, 37 infants in the first 3 months of life underwent open aortic valvotomy for critical aortic stenosis. All patients underwent cardiopulmonary bypass, valvotomy, and valve debridement under direct vision with standard techniques. RESULTS: Early mortality was 11% (4 of 37, 70% confidence limit 7% to 20%) and all early deaths were in neonates less than 2 weeks of age. Late death occurred in 6 patients a mean of 10 +/- 12 months (range, 2 to 36 months) after valvotomy. Actuarial survival, including operative deaths was 92% +/- 6% at 1 month, 78% +/- 9% at 1 year, and 73.4% +/- 10% at 10 years. In a multifactorial regression analysis, the best predictors of death were the presence of endocardial fibroelastosis and small body surface area and the best predictor of the need for late reintervention was preoperative aortic annular size. Thirteen patients required reintervention: repeat operation in 7 patients, balloon valvuloplasty in 3 patients, and both balloon valvuloplasty and reoperation in 3 patients. Actuarial freedom from reintervention postoperatively is 97% +/- 3% at 1 month, 73% +/- 9% at 1 year, and 55% +/- 11% at 10 years. Reintervention was for recurrent left ventricular outflow obstruction in 9 patients and mixed aortic stenosis and aortic insufficiency in 4. Echocardiography 4.3 +/- 2.5 years after aortic valvotomy in survivors who have not required reintervention (n = 20) revealed a Doppler peak instantaneous systolic gradient of 37 +/- 14 mm Hg and mild or less aortic regurgitation in 16 patients and moderate aortic regurgitation in 4 patients. CONCLUSIONS: Current surgical results with critical aortic stenosis in the neonate and young infant are acceptable in terms of both late survival, reintervention, and functional results in the majority of patients. Newer interventions, such as balloon valvuloplasty, should be carefully evaluated for long-term results and should be compared more appropriately to current surgical results to determine the best treatment modality for the neonate and infant with critical aortic stenosis.

Actuarial Analysis↗