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Metastatic hepatocellular carcinoma obstructing the right ventricular outflow tract.

A 49-year-old female with a past history of liver resection due to hepatocellular carcinoma was referred to our Department for treatment of a metastatic cardiac tumor obstructing the right ventricular outflow tract. She underwent operation twice with cardiopulmonary bypass, and symptoms were relieved. Metastasis from hepatocellular carcinoma to the heart is very rare, but should be taken into consideration during follow-up after treatment for a primary liver tumor.

Carcinoma, Hepatocellular↗

A new technique of arterial switch operation with in situ coronary reallocation for transposition of great arteries.

Coronary artery translocation is the most important step in achieving a successful result in arterial switch operations. Although a few centers have reported excellent results, coronary artery transfer requires a high technical expertise. We report a new technique of arterial switch operation without coronary translocation. By creating flaps in the proximal great arteries, the coronaries are transferred to the neoaorta without distortion of their original anatomic position. This technique avoids problems related to coronary translocation. Because coronary buttons are not excised, there is no need for nonviable material to be used in reconstruction of neopulmonary artery. Arterial wall is sutured to arterial wall, so postoperative bleeding is lessened. This technique can be used for any type of coronary anomaly and great arterial relationship. Coronary perfusion is well maintained. Two patients with transposition variants and ventricular septal defects have been operated on successfully with this technique. Postoperative investigations showed good coronary perfusion, without right or left ventricular outflow obstruction or leakage through the semilunar valves. This technique achieves anatomic correction for transposition of the great arteries, just as a conventional arterial switch operation does, but it avoids problems related to coronary artery translocation. We believe that it is a much simpler, more reliable, and more reproducible operation than others in current use, and it can be carried out by many cardiac surgeons with acceptable results. The early results are encouraging, although longer follow-up and more cases are essential.

Cardiac Surgical Procedures↗

Sex-associated differences in left ventricular function in aortic stenosis of the elderly.

BACKGROUND: In aortic stenosis, the response of the left ventricle to pressure overload varies from compensated hypertrophy to overt heart failure. The determinants of left ventricular adaptation are poorly understood. METHODS AND RESULTS: Left ventricular function was compared to assess the role of sex in 34 women and 29 men 60 years or older with both hemodynamic and echocardiographic data characteristic of severe aortic stenosis and no important coronary artery disease. Despite a similar degree of left ventricular outflow obstruction in women versus men (aortic valve area 0.54 +/- 0.20 versus 0.59 +/- 0.19 cm2, NS), the left ventricle of women had a greater fractional shortening (37 +/- 12 versus 25 +/- 12%, p = 0.001), achieved a smaller end-systolic chamber size (1.82 +/- 0.64 versus 2.17 +/- 0.65 cm/m2, p = 0.04), and generated more pressure (210 +/- 35 versus 182 +/- 29 mm Hg, p = 0.001) with a greater maximum positive dP/dt (2.153 +/- 794 versus 1,595 +/- 384 mm Hg/sec, p = 0.02). The men had a lower cardiac index (2.12 +/- 0.59 versus 2.49 +/- 0.63 l/min/m2, p = 0.02), higher mean pulmonary artery pressure (35 +/- 13 versus 27 +/- 10 mm Hg, p = 0.01), and shorter ejection period (340 +/- 40 versus 370 +/- 40 msec, p = 0.02). Women and men were equally symptomatic. Supernormal left ventricular ejection performance was present in 41% of the women and only 14% of the men (p = 0.002). This subgroup of women had a small, thick-walled chamber (end-diastolic radius to thickness ratio, 1.58 +/- 0.52 versus 2.45 +/- 0.51 in control women, p = 0.01) with low end-systolic wall stress. Subnormal ejection performance was present in 64% of the men and only 18% of the women (p = 0.002). This subgroup of men had an increased chamber size and high end-systolic wall stress compared with control men. Greater left ventricular mass was present in men compared with women (211 +/- 55 versus 179 +/- 55 g/m2, p = 0.03). CONCLUSIONS: Sex is a factor in left ventricular adaptation to valvular aortic stenosis in adults 60 years or older.

Aged↗

[Secondary atypical hypertrophy: hypertrophic cardiomyopathy with acquired risk factors (author's transl)].

New clinical concept of "secondary atypical hypertrophy" was proposed for hypertrophic cardiomyopathy (HCM) associated with an acquired risk factors such as hypertension, strenuous exercise, etc, based on the following findings. 1) Case-control study suggested that history of hypertension, physical labor and weight gain were thought to be risk factors of apical hypertrophy. History of hypertension was also demonstrated in 55% of older cases of HCM (greater than 35 yrs) with asymmetric septal hypertrophy, and this figure was appreciably higher than that in general population (around 26% in our population survey). Thus hypertension was suggested to have a causal relationship to HCM as an important risk factor in older cases. 2) Even in cases without hypertension, 29% of non-obstructive HCM exhibited a marked increase in systolic blood pressure on bicycle ergometer stress test, suggesting an important of transient hypertension during exercise as a risk factor of unusual hypertrophy. 3) The cases of HCM with definite family history and the cases of HCM with secondary atypical hypertrophy, with acquired risk factors such as hypertension, presented different clinical features. The latter is older and predominant in males. In the former, QRS pattern was les distorted, suggesting milder congenital defect of the myocardium. Left ventricular function was less impaired in cases with secondary atypical hypertrophy if judged from diastolic descent rate of the mitral valve, left ventricular enddiastolic pressure and functional aerobic impairment (FAI). On the other hand, no differences were observed between cases with ventricular septal hypertrophy and left ventricular outflow obstruction. 4) Follow-up study demonstrated rather favorable prognosis in cases with secondary atypical hypertrophy because of the absence of sudden death despite of their older age. These distinctive difference in clinical features, prognosis and then management in cases with HCM with acquired risk factors suggested a validity of the concept of secondary atypical hypertrophy in clinical practice.

Adult↗

False diagnosis of subpulmonary obstruction by echocardiography in d-transposition of the great arteries.

Twenty-one consecutive children (aged 3 to 14 months) with d-transposition of the great arteries were evaluated with standard M mode echocardiography at the time of hospital admission for routine preoperative cardiac catheterization. At catheterization, 6 of the 21 were found to have a systolic pressure gradient between the body of the left ventricle and the main pulmonary artery. Echocardiographic systolic anterior mitral valve motion was noted in nine patients, including five who did not have a left ventricular to pulmonary arterial systolic pressure gradient. Diastolic approximation of the anterior mitral leaflet to the septum was noted in 14 (9 without a gradient), pulmonary valve flutter was also present in 14 (9 without a gradient) and there was premature systolic pulmonary valve closure in 6 (3 without a gradient). Echographic left heart dimensions were not different in patients with a left ventricular outflow pressure gradient than in those without, but patients wit systolic anterior mitral valve motion and diastolic approximation of the mitral leaflet to the septum did have smaller left ventricular and pulmonary root dimensions than did those without these echocardiographic findings. Echocardiographic findings that have been described as suggesting left ventricular outflow obstruction in patients with d-transposition of the great arteries appear to be more related to size and configuration of the left ventricle than to the presence or absence of obstruction.

Cardiac Catheterization↗

Management after childhood repair of tetralogy of fallot.

The four characteristic findings in tetralogy of Fallot (TOF) include the following: 1) a malaligned ventricular septal defect; 2) right ventricular outflow and/or pulmonary valve/artery stenosis or atresia; 3) a dextraposed overriding aorta; and 4) right ventricular hypertrophy. This article focuses solely on the outcomes and treatment of surgically corrected patients born with TOF. In the modern era, early surgical repair consisting of ventricular septal defect closure and alleviation of right ventricular outflow obstruction have gained favor over early palliation with an aortopulmonary shunt followed by intracardiac repair. Surgical outcomes are excellent and dramatically improve prognosis. However, these patients are not "cured" and are at significant risk of developing subsequent electrical and hemodynamic problems. An operated patient with TOF should be evaluated at regular intervals by a cardiologist trained in congenital heart disease; any symptoms suggestive of hemodynamic or electrical compromise should spur further investigation. Advances in imaging, medical therapy, electrophysiology, device/resynchronization therapy, and percutaneous intervention provide the clinician with a number of therapeutic options. Surgical techniques have changed significantly since the early intracardiac repairs of the 1960s and 1970s. The deleterious hemodynamic and electrical effects of pulmonary regurgitation and ventriculotomy scars have spurred efforts to ensure pulmonary valvular competence and minimize the extent of ventricular incisions. Since Blalock and Taussig's first palliative shunt in 1945, the survival and quality of life for patients with TOF has improved dramatically; this is one of the great accomplishments for cardiovascular medicine in the 20th century. The 21st century promises further success with the advent of a myriad of technologic advancements.

Journal Article↗

External carotid pulse recordings in hypertrophic obstructive cardiomyopathy.

External carotid pulse tracings were examined in 15 patients with hypertrophic obstructive cardiomyopathy (HOCM), the diagnosis being confirmed by catheterization of the left heart. Of 12 patients with intraventricular gradients at rest, 9 had a typical bifid pulse with midsystolic dipping. In one patient without a gradient at rest, midsystolic dipping occurred only in beats following extrasystoles (Brockenbrough phenomenon). The upstroke of the pulse wave was rapid in all the patients, a finding that distinguishes them from patients with valvular aortic stenosis. There was a correlation between the length of the left ventricular ejection time and the intraventricular gradient (r = 0.71) but as more than half the patients had normal or shortened ejection times, the diagnosis of HOCM cannot be based on measurements of this parameter. It is concluded that carotid pulse registrations are of considerable diagnostic value in patients suspected of having HOCM. As the pulse changes are correlated to the degree of left ventricular outflow obstruction, it is suggested that repeated pulse tracings may be used as a means for controlling the degree of obstruction once the diagnosis has been established in the individual patient.

Adult↗

Left ventricular inflow obstruction associated with persistent left superior vena cava and dilated coronary sinus.

BACKGROUND: It has previously been suggested that significant dilatation of the coronary sinus can contribute to left ventricular inflow obstruction and is amenable to surgical correction. The purpose of this study was to review our experience with this rare condition. METHODS: Since 1995, 6 patients have undergone coronary sinus reduction for concerns of obstruction with other concomitant intracardiac repairs. Preoperative echocardiography identified a significantly dilated left superior vena cava to the coronary sinus in 5 patients (83%) and an abnormal mitral valve in 4 patients (67%); these resulted in abnormal Doppler inflow patterns. Preoperative cardiac catheterization was performed in 5 patients and revealed increased atrial "a" waves, with a gradient to the left ventricular end-diastolic pressure in each case. At the time of surgery, coronary sinus angioplasty was performed in all patients. RESULTS: There were no deaths, and there was no major morbidity. Follow-up imaging revealed no significant left ventricular inflow obstruction in any patient. CONCLUSIONS: We conclude that dilatation of the coronary sinus can become hemodynamically significant and that coronary sinus angioplasty is a safe and effective technique.

Adolescent↗

Right ventricular metastatic choriocarcinoma obstructing inflow and outflow tract.

We operated on a 34-year-old man with a metastatic tumor that extended from the tricuspid valve to the pulmonary valve and obstructed the right ventricle inflow and outflow tracts. The tumor was removed with preservation of the tricuspid valve. Additional chemotherapy was carried out according to the BEPO (etoposid, eisplatin, bleomycin, vincritin) scheme. Histology revealed metastasis of a choriocarcinoma originating from the right testis. Computed tomography performed after 6 months detected no metastases in the lungs. Magnetic resonance imaging showed a thickened right ventricle free wall and apex. The patient is doing well 18 months postoperatively.

Adult↗

[Quantitative evaluation of right heart obstructions in children using Doppler echocardiography].

To assess the accuracy of pulsed and continuous-wave Doppler echocardiography (DE) for estimating pressure drops across right ventricular outlet obstruction, we examined 27 children (valvular pulmonary stenosis n = 16, complex congenital heart lesion with prior pulmonary banding n = 11). The Doppler evaluation was performed within 24 hours of cardiac catheterization in 17 patients, in 10 it was done 2-24 months (mean 12.2 months) after catheterization. We found a good correlation (r = 0.93) with slight underestimation of pulmonary valve gradient. Using three windows (left parasternal, suprasternal and subcostal) the ultrasound beam can be better aligned parallel to the excentric jet of a malformed valve than using the left parasternal position alone. In children with complex congenital heart disease and normally related great arteries Doppler ultrasound reliably estimates the gradient across a pulmonary artery band using the left parasternal transducer position (r = 0.92). In patients with malposition of the great arteries the use of multiple windows is necessary. Thus, DE provides an accurate noninvasive method of estimating pressure gradients in patients with right ventricular outflow obstructions and is helpful in determining the subsequent clinical management.

Blood Pressure↗

Two dimensional echocardiography in congenital heart disease.

Two dimensional echocardiography has enhanced the ability of the clinician to define accurately intracardiac anatomy and great vessel relations. By visualizing relations of the great arteries and determining ventricular situs in the parasternal short axis planes and by assessing the relation of the great arteries to ventricles atrioventricular (A-V) valves and the interventricular septum in the parasternal and apical long axis view, complex congenital heart disorders can readily be differentiated. The subcostal approach allows accurate identification and localization of interatrial and interventricular septal defects. Utilizing the apical parasternal four chambered or subcostal four chambered view, complex malformations of the A-V valves such as complete endocardial cushion defect and Ebstein's anomaly can readily be appreciated. The suprasternal approach has allowed direct visualization of a coarcted aortic segment. The differentiation of left and right ventricular outflow obstruction is also possible. In some patients, it is possible to achieve an accurate diagnosis with two dimensional echocardiography alone. In others, two dimensional echocardiography provides accurate and detailed information with regard to spatial anatomy and valve morphology so that invasive studies can be performed more expeditiously, with less contrast agent and hence with greater safety. With regard to future developments, better resolution capability with newer instrumentation and combined two dimensional echocardiography with Doppler blood flowmeter studies may provide even greater diagnostic accuracy in the evaluation of patients with congenital heart disorders.

Aorta↗

[Right mid-ventricular stenosis with intact interventricular septum].

Pure infundibular stenosis of the right ventricle is a rare congenital anomaly. Right midventricular stenosis with intact interventricular septum is even rarer. It consists of obstruction of the trabecular zone of the right ventricle by a hypertrophied moderator band with or without abnormal muscle bundles. This anomaly can now be diagnosed by transthoracic echocardiography, while haemodynamic and angiographic diagnosis is reserved for doubtful cases. Two cases of midventricular stenosis are reported. In both patients, the diagnosis was suspected bu ultrasonography which showed a right intraventricular gradient of about 80 mmHg without identifying the exact site (infundibular or midventricular). The diagnosis was corrected by the operative findings. A good postoperative course was observed for both patients.

Adolescent↗

Tetralogy of Fallot with absent pulmonary valve. Should valve insertion be part of the intracardiac repair?

Thirteen patients with complex of tetralogy of Fallot and absent pulmonary valve were treated. Three infants presented with severe cardiorespiratory distress (Group I) necessitating surgical intervention in two and intensive medical treatment in the third; there were no late survivals. The remaining 10 patients were operated upon electively at an older age (Group II); in six the ventricular septal defect was closed and the right ventricular outflow obstruction relieved (Group IIA), and in the other four a valve was inserted at the pulmonary anulus at the time of intracardiac repair (Group IIB). There was no hospital mortality in Group II. Five of the six patients in Group IIA remained symptomatic postoperatively, and three needed a subsequent pulmonary valve insertion. All patients in Group IIB were symptom free following operation; one patient began having a progressive decrease in exercise performance, decrease in pulmonary artery size, and cardiothoracic ratio were more marked in those patients who had primary insertion of a pulmonary valve. The clinical results and postoperative assessment of cardiac function tend to indicate that pulmonary valve insertion should be performed at the time of the intracardiac repair.

Adolescent↗

Total percutaneous correction of a tetralogy of Fallot variant with dominant pulmonary valve stenosis.

OBJECTIVE: To study the feasibility, efficacy, and safety of total percutaneous correction of a tetralogy of Fallot variant with dominant pulmonary valve stenosis. DESIGN: Percutaneous correction of a variant of tetralogy of Fallot with dominant pulmonary valve stenosis, on the basis that there are transcatheter methods for the correction of malalignment-type ventricular septal defect (VSD) (transcatheter patch) and valvar pulmonary stenosis (balloon valvoplasty). PATIENTS: Two patients with tetralogy of Fallot, 4 and 7 years old, were admitted for percutaneous correction. Their aortic saturations were 72% and 88%. Both had severe right ventricular outflow obstruction with dominant valvar pulmonary stenosis with total gradients of 120 and 70 mm Hg. Large malalignment subaortic VSDs, 14 and 16 mm in diameter, were present. The first patient had a previous percutaneous correction of a small atrial septal defect and an aortopulmonary collateral. INTERVENTIONS: Balloon valvoplasty was first performed, followed by balloon test occlusion of the VSD and double balloon patch occlusion. Forty eight hours after implantation the supporting balloons were extracted, releasing the patches. RESULTS: Both patients became acyanotic with oxygen saturations of 96%. There was mild residual infundibular stenosis with 40 and 30 mm Hg gradients. Both VSDs were effectively occluded with only trivial residual shunts. One patient developed mild haemolysis, which resolved spontaneously in a few days. Both patients were doing well at six and 12 months' follow up visits. CONCLUSIONS: Total percutaneous correction of the tetralogy of Fallot variants with dominant pulmonary valve stenosis is feasible and successful. Larger clinical trials are required to further assess effectiveness and safety.

Catheterization↗

Polypoid sarcoma of the pulmonary trunk: analysis of the literature and report of a case with leptomeric organelles and ultrastructural features of rhabdomyosarcoma.

A case of polypoid sarcoma of the pulmonary trunk is described in an 80-year-old woman who had a 21-year history of episodic chest pain and hemoptysis. Ultrastructural examination revealed Z bands, characteristic of rhabdomyosarcoma, and leptomeric organelles. This is the 60th reported case of sarcoma of the pulmonary trunk. Symptoms in reported cases were variable, usually caused by tumor emboli to the lungs or by right ventricular outflow obstruction. Angiography was diagnostic. The tumor was characteristically polypoid, often multicentric, and by definition was fixed to the pulmonary trunk or valves. Non-differentiated sarcoma (often uniquely pleomorphic) was found in 37% of patients, leiomyosarcoma (once confirmed by electron microscopy) in 17%, myxosarcoma in 13%, elements of rhabdomyosarcoma in 8%, fibrosarcoma in 8%, elements of chondrosarcoma in 11%, and 5% were malignant mesenchymomas. The authors suggest that these tumors originate from the undifferentiated tissues of the bulbus cordis and propose the name myenchymoma for the tumor.

Adult↗

Evaluation of alprostadil (prostaglandin E1) in the management of congenital heart disease in infancy.

Prostaglandins have been shown to relax the smooth muscle of the ductus arteriosus in the fetus in utero. This physiologic action has been applied to the management of newborn infants with certain types of congenital malformations. Infants with lesions producing right ventricular outflow obstruction have a compromised pulmonary circulation and require a patent ductus arteriosus for adequate pulmonary blood flow. Infusion of alprostadil (PGE1) dilates the ductus, increases pulmonary blood flow, and thereby improves oxygenation. Likewise, infants with aortic arch interruption or coarctation of the aorta are dependent on an open ductus to maintain lower body perfusion. Alprostadil is of great benefit in this situation as well. The side effects of alprostadil include peripheral vasodilation and hypotension and, most importantly, apnea. Hyperpyrexia and jitteriness may also occur. Side effects occur only in about 20% of infants and usually are easily reversed. The benefits therefore greatly outweigh the risks, but careful monitoring is essential.

Alprostadil↗

Polysplenia: a review of 146 cases.

The types of cardiac and visceral anomalies of 146 autopsied cases of polysplenia are described. One hundred and five of these cases were from the literature and the other 41 cases were specimens we personally reviewed. The anomalies found support the previous description of this condition as bilateral leftsidedness. Each lung had 2 lobes in 55% of patients, and abdominal heterotaxia was present in 56%. Cardiac anomalies occurring in at least half of the patients include bilateral superior vena cava, interruption of the inferior vena cava with azygos continuation, ventricular septal defect, ostium primum defect, and morphologic left ventricular outflow obstruction. Not all patients with polysplenia have cardiac anomalies, and in many patients the cardiac anomalies are not complex. Fifty percent of the 146 patients died by 4 months of age and 75% before 5 years of age.

Abnormalities, Multiple↗

Primary pulmonary artery sarcoma in two children.

The clinical and pathological features of primary pulmonary artery sarcoma in two children are reported. The first patient presented with right ventricular outflow obstruction and underwent successful surgical resection of his tumor. The second patient developed cardiac arrest following a relatively short period of symptoms of right heart failure and could not be revived. The pathological diagnosis in both patients was hemangiopericytoma. To the best of our knowledge, primary pulmonary artery sarcoma in children has not been previously reported.

Adolescent↗