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Results of the Senning procedure in infants with simple and complex transposition of the great arteries.

Because of interest in the arterial switch operation, recent experience with the Senning operation in patients with simple and complex transposition of the great arteries (TGA) was examined. Between 1982 and 1985, 35 patients with simple TGA (group I) and 10 patients with complex TGA (group II) underwent a Senning operation. Mean duration of follow-up was 14 months for group I and 24 months for group II. In group I, 1 patient died early and no patient died late; infrequently, right ventricular dysfunction, tricuspid regurgitation, baffle obstruction or arrhythmias occurred. In group II, no patient died early and 3 died late. In addition, many patients required prolonged digoxin therapy. Because the arterial switch operation has a high early mortality risk and an undetermined long-term morbidity and mortality risk, the Senning operation (or other venous switch operations) is considered the preferred surgical approach for simple TGA. In contrast, an arterial switch operation is performed in patients with complex TGA (without subpulmonic obstruction). Comparison of the early and late morbidity and mortality of the arterial vs the venous switch operation may be helpful in selecting the appropriate surgical approach to complex TGA.

Female↗

Intravenous isosorbide dinitrate during open-heart surgery and its role in the treatment of right-sided congestive heart failure.

Recent awareness of the importance of the functional integrity of the right ventricle and the effect of raised pulmonary vascular resistance on cardiac output after cardiopulmonary bypass has focused attention on means of protecting right ventricular myocardium and reducing right ventricular afterload during open-heart surgery. A study of the acute effects of bolus intravenous isosorbide dinitrate (ISDN) has shown that after cardiopulmonary bypass, bolus intravenous ISDN produced highly significant (p less than 0.001) decreases in mean pulmonary arterial pressure (13%), pulmonary vascular resistance (23%) and the ratio of pulmonary to systemic vascular resistance (20%), indicating that active pulmonary vasodilation had occurred in the absence of other hemodynamic changes. The results suggest that possibly the acute effect of low-dose ISDN after cardiopulmonary bypass is predominantly exerted on the right ventricular afterload if systemic arterial pressure is not elevated. Two different clinical situations are described in which intravenous ISDN proved beneficial, one being acute pulmonary hypertension after protamine sulphate and the second being acute right-sided congestive heart failure with systemic hypotension unresponsive to conventional therapeutic measures. Thus, ISDN may prove a useful agent for alleviating right ventricular dysfunction at a time of not infrequent cardiovascular instability, the period after bypass.

Cardiac Surgical Procedures↗

Myocardial function and perfusion in the CREST syndrome variant of progressive systemic sclerosis. Exercise radionuclide evaluation and comparison with diffuse scleroderma.

Myocardial function and perfusion were evaluated in 22 patients with progressive systemic sclerosis with the CREST syndrome using exercise and radionuclide techniques, pulmonary function testing, and chest roentgenography. The results were compared with a similar study of 26 patients with progressive systemic sclerosis with diffuse scleroderma. The prevalence of thallium perfusion abnormalities was similar in the groups with CREST syndrome and diffuse scleroderma, (64 percent versus 77 percent), but the defects were significantly smaller in the CREST syndrome (p less than 0.01). Reperfusion thallium defects in the absence of extramural coronary artery disease were seen in 38 percent of patients with diffuse scleroderma. This finding was not seen in any of the patients with the CREST syndrome. In diffuse scleroderma, abnormalities of both right and left ventricular function were related to larger thallium perfusion defects. In the CREST syndrome, abnormalities of left ventricular function were minor, were seen only during exercise, and were unrelated to thallium perfusion defects. Abnormal resting right ventricular function was seen in 36 percent of the patients with the CREST syndrome and was associated with an isolated decrease in diffusing capacity of carbon monoxide. It is concluded that the cardiac manifestations of the CREST syndrome are distinct from those found in diffuse scleroderma. Unlike diffuse scleroderma, abnormalities of left ventricular function in the CREST syndrome are minor and are unrelated to abnormalities of coronary perfusion. Right ventricular dysfunction in the CREST syndrome appears to be primarily related to pulmonary vascular disease.

Adult↗

Stage I palliation of hypoplastic left heart syndrome: the importance of neoaorta construction.

After Norwood's initial report of successful first-stage palliation of hypoplastic left heart syndrome in neonates, the occurrence of distal aortic obstructions, shunt problems, and late deaths have led to modifications in the surgical technique. Between January 1986 and December 1987, 12 neonates from three to 16 days old underwent stage I palliation with the same objectives. An open atrial septectomy was always performed. The pulmonary artery bifurcation was transected from the main pulmonary artery and closed with an aortic homograft patch. The aortotomy was begun 2 cm below the patent ductus arteriosus insertion and extended across the transverse arch and down the ascending aorta. The neoaorta was constructed using the hypoplastic ascending aorta-transverse aortic arch, the main pulmonary artery, and an aortic homograft augmentation patch. The homograft is hemostatic and pliable, and molds well in forming the neoaorta. A 4-mm shunt was inserted between the right innominate artery and the right pulmonary artery in 5 patients and the neoaorta and the pulmonary artery bifurcation patch in 7 patients. The early systemic oxygen saturation was optimized at 75% to 80% with hyperventilation, high concentration of inspired oxygen, sodium bicarbonate, and the frequent use of vasopressors to maintain an arterial blood pressure of 65 to 75 mm Hg. Two patients (17%) died early after operation; 1 had severe right ventricular dysfunction and both had severe tricuspid regurgitation. There were 2 late deaths at 7 and 13 months, of sepsis and hypoxia. The 8 survivors (67%) continue to do well over follow-up. The preoperative tricuspid regurgitation has remained stable in 3 survivors and disappeared in 2 survivors.(ABSTRACT TRUNCATED AT 250 WORDS)

Aorta↗

Transesophageal echocardiography in patients with mechanical circulatory assistance.

Transesophageal echocardiography was used to assess myocardial function and to detect complications after mechanical circulatory support for 8 patients with cardiogenic shock. In 3 of 8 patients, serial transesophageal echocardiography documented improvement of systolic ventricular function, and it was possible to wean these 3 patients from the ventricular assist device. In all patients, transesophageal echocardiography added clinically important information including the extent of left and right ventricular dysfunction (6 patients), presence of atrial or ventricular thrombus (5 patients), presence of pericardial effusion or clot (2 patients), and verification of the position of the intravascular device (1 patient). Thus, transesophageal echocardiography may provide clinically useful information regarding both the underlying cardiac disease and potential complications from the mechanical circulatory assistance.

Adult↗

Long-term results after atrial correction of complete transposition of the great arteries.

This study presents the late results for the first 104 consecutive patients surviving and atrial repair for transposition of the great arteries (TGA) between January 1971 and December 1978 (group 1). Mean follow-up was 12 years (range, 0.1 to 17.7 years). The actuarial survival rate at 18 years was 84.2% (70% confidence limits, 79% to 88%) for simple TGA and 93.7% (70% confidence limits, 84% to 97%) for complex TGA. Nine of the 11 deaths were sudden. Two (2.6%) of the 78 late survivors operated on for simple TGA are in New York Heart Association functional class III or IV versus 4 (26.7%) of the 15 survivors with complex TGA; the other patients are doing very well. To better assess long-term results, we report the findings for randomly obtained electrocardiograms, Holter monitor recordings, radionuclide angiographic studies, and cardiac catheterizations performed in 1987 in a larger group of 159 long-term survivors of atrial repair operated on at Ospedale Riuniti di Bergamo from January 1971 to December 1984 (group 2), which includes all of group 1. The findings confirm that the arterial switch repair is the procedure of choice for complex TGA and that there is a major incidence (approximately 10%) of systemic right ventricular dysfunction and rhythm disturbances after the atrial repair. On the other hand, our late survival rate at 18 years of 84% for simple TGA with 97.5% of the patients in functional class I is a result that should be kept in mind, especially in institutions where the arterial switch is a relatively new approach and presumably is a higher risk to cause early death.

Cardiac Catheterization↗

Absent pulmonary valve syndrome: operation in infants with airway obstruction.

From 1979 through 1991, 19 infants with absent pulmonary valve syndrome and airway obstruction were seen for surgical treatment. All patients underwent extensive pulmonary artery aneurysmorrhaphy using cardiopulmonary bypass. Fourteen patients had simultaneous transatrial ventricular septal defect (VSD) closure, infundibular resection, and placement of a short transannular patch; 2 had transventricular VSD closure and infundibular resection without a transannular patch; 1 underwent transventricular VSD closure and transannular patching; and 2 underwent pulmonary artery aneurysmorrhaphy alone with the VSD left open. All 19 infants had good hemodynamics when taken from the operating theater, but 3 died postoperatively of severe airway obstruction, despite further tracheobronchopexy procedures in 2 (hospital mortality rate, 16%; confidence limits, 7% to 29%). Among the 16 patients discharged from the hospital, there was one late death. Five other patients have required reoperation for branch pulmonary artery stenosis (n = 2), residual airway obstruction resulting from persistent pulmonary artery dilatation (n = 1), closure of VSD (n = 1), and homograft valve insertion for pulmonary incompetence and right ventricular dysfunction (n = 1). There are 15 long-term survivors. Eight of them have episodic bronchospasm of mild to moderate severity, and all are responsive to sympathomimetic bronchodilator aerosols. The remaining 7 are asymptomatic.

Airway Obstruction↗

Transatrial repair of ventricular septal defect subsequent to myocardial infarction.

We describe successful transatrial repair of a ventricular septal defect occurring secondary to myocardial infarction. This approach avoids the need for ventriculotomy and may be technically simpler and safer than repair across the zone of infarction in a subset of patients with posterior defects and right ventricular dysfunction who survive the early period subsequent to their infarct.

Aged↗

Abnormalities of pulmonary function in congestive heart failure.

The heart and the lungs are strictly integrated both mechanically and functionally in what is now called the cardiopulmonary unit [1-4]. As a consequence, an abnormality of either component of this cardiopulmonary unit quickly alters the physiology of the other. In fact, right ventricular dysfunction and failure secondary to lung disease (i.e. cor pulmonale) are well known; on the other hand the impairment of lung function induced by left heart failure seems obvious, but the knowledge of its pathophysiological mechanism is still incomplete. In this article we will review the structural and functional changes induced by congestive heart failure on the lungs, their relation to the hemodynamic abnormalities and response to therapeutic interventions.

Heart Failure↗

Restrictive left ventricular filling pattern in dilated cardiomyopathy assessed by Doppler echocardiography: clinical, echocardiographic and hemodynamic correlations and prognostic implications. Heart Muscle Disease Study Group.

OBJECTIVES: This study was undertaken to evaluate the frequency of restrictive left ventricular filling pattern in dilated cardiomyopathy, as well as its clinical and hemodynamic correlations and prognostic implications. BACKGROUND: In dilated cardiomyopathy, as in other heart diseases, different left ventricular filling patterns were observed on Doppler echocardiography. Some patients showed a "restrictive filling pattern," similar to that associated with restrictive cardiomyopathy, characterized by predominant E waves and a shortened E deceleration time. METHODS: Pulsed Doppler transmitral curves were analyzed in 79 consecutive patients with dilated cardiomyopathy assigned to two study groups according to E deceleration time: group 1 (n = 36) had a restrictive left ventricular filling pattern (E deceleration time < 115 ms); group 2 (n = 43) had an E deceleration time > or = 115 ms. RESULTS: Patients in group 1 were significantly younger, in a higher New York Heart Association functional class, more frequently had a third heart sound and had a higher left ventricular filling pressure at catheterization. In addition, they showed more severe left and right ventricular dysfunction and dilation, a larger left atrium and more severe mitral regurgitation. A restrictive filling pattern was associated at Doppler study with a higher E wave velocity, lower A wave velocity and higher E/A ratio. During a follow-up interval of 22 +/- 14 months, all 14 patients who subsequently died or required heart transplantation showed a restrictive left ventricular filling pattern. At multivariate analysis, E deceleration time was the most powerful independent prognostic indicator of poor outcome or transplantation. CONCLUSIONS: Restrictive left ventricular filling pattern is frequent in dilated cardiomyopathy, is associated with more severe disease and is a powerful indicator of increased mortality risk and need for heart transplantation.

Adolescent↗

Primary pulmonary hypertension in children: clinical characterization and survival.

OBJECTIVES: This study characterized mortality in a group of Mexican children (n = 18, mean [+/- SD] age 9.9 +/- 3 years) with primary pulmonary hypertension and investigated the factors associated with their survival. BACKGROUND: Primary pulmonary hypertension is a progressive, fatal disease of unknown cause. Establishing the diagnosis earlier in life may influence prognosis. METHODS: A dynamic cohort of children with primary pulmonary hypertension were enrolled between December 1977 and May 1991 and followed up through September 1992. Measurements included hemodynamic and pulmonary function variables in addition to demographic data, medical history and response to vasodilator treatment. We also compared the survival estimates of these children with those of our adult patients with primary pulmonary hypertension (n = 42, mean age 27.9 +/- 8.5 years). RESULTS: Baseline mean (+/- SD) pulmonary artery pressure was similar in children and adults (66 +/- 15 vs. 65 +/- 18 mm Hg, p = NS), but a higher cardiac index resulted in a lower mean pulmonary vascular resistance index in children (18 +/- 7 vs. 26 +/- 12 U/m2, p < 0.01). The proportion of patients who had a positive hemodynamic response to vasodilator treatment was higher in children than in adults (41% vs. 25%). Estimated median survival in children was 4.12 years (95% confidence interval [CI] 0.75 to 8.66) and 3.12 years in adults (95% CI 0.5 to 13.25, chi-square log-rank 0.81, p = NS). Elevated right atrial pressure (rate ratio 10.2) and decreased stroke volume index (rate ratio 32.9) were the only significant predictors of mortality (Cox proportional hazards model). CONCLUSIONS: Children with primary pulmonary hypertension have a poor survival expectancy, which does not appear to differ from that in adults with primary pulmonary hypertension. Mortality in childhood primary pulmonary hypertension is also associated with variables that assess right ventricular dysfunction.

Adult↗

Surgical management of complete atrioventricular canal associated with tetralogy of Fallot.

Between 1984 and 1993, 12 children with an atrioventricular canal and tetralogy of Fallot underwent surgical repair. The mean(s.d.) age at operation was 58(18) months, and the mean(s.d.) body weight 15(4) kg. Nine patients underwent 11 palliative procedures. The ventricular septal defect was closed through a combined (right atrial and right ventricular) approach in nine cases, and through a right atrial approach in three, using a prosthetic patch with a wide anterior extension, secured with a running suture. The 'ostium primum' defect was closed with a separate prosthetic patch in 11 cases (double-patch technique). Right ventricular outflow obstruction was relieved by a composite infundibular patch (seven cases) or a transanular patch (five). There were four hospital deaths (33%). These were caused by low cardiac output in three cases and infection in one (three deaths occurred in patients with a transanular patch). One patient has so far died during follow-up. Assessment at 50(36) months by echo-Doppler showed moderate-to-severe 'mitral' regurgitation in three cases, and moderate 'tricuspid' regurgitation with right ventricular dysfunction in one case. Two patients have required further surgery.

Blood Vessel Prosthesis↗

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Adult↗

Surgical treatment of absent pulmonary valve syndrome associated with bronchial obstruction.

BACKGROUND: Absent pulmonary valve syndrome (APVS) is a relatively rare anomaly that is usually associated with a ventricular septal defect and a restrictive pulmonary annulus with severe pulmonary regurgitation. The to-and-fro flow across the right ventricular outflow tract produces enormously dilated and pulsatile pulmonary arteries (PAs) that cause severe respiratory distress and tracheomalacia by compression of the trachea and primary bronchi. This retrospective study summarizes our 20-year experience of surgical treatment in patients with APVS. METHODS: Between 1984 and 2005, 20 patients underwent repair of APVS using a valved conduit (n = 9), transannular patch (TAP) insertion alone (n = 5), or TAP with monocusp valve (n = 6) with PA reduction arterioplasty. Median age was 7 months (range, 6 days to 9 years). RESULTS: There were one perioperative and two late deaths. All deaths were related to airway complications. Survival was 90% at 1 year and 85% at 10 and 15 years. In a multivariate analysis only preoperative ventilator dependency was associated with a worse outcome (p = 0.02). Follow-up was available in 18 operative survivors (mean follow-up, 7.3 +/- 5.2 years). Six patients (33%) underwent reoperation for pulmonary valve incompetence and right ventricular dysfunction. Three patients (17%) had episodic bronchospasm of mild to moderate severity that were responsive to sympathomimetic bronchodilators. CONCLUSIONS: Morbidity associated with perioperative respiratory complications and ventilator dependency due to underlying tracheobronchomalacia is an important problem in patients with APVS. These infants may require multiple hospitalizations for recurrent respiratory infections secondary to their tracheobronchomalacia. Complete repair with a valved conduit and reduction pulmonary arterioplasty at the onset of symptoms and a definitive diagnosis is our procedure of choice for infants with APVS. With this approach, the airway can be optimized to give the best patient outcome.

Airway Obstruction↗

Pulmonary hypertension.

Pulmonary hypertension, in its simplest sense, is elevation of the pulmonary artery pressure above normal. A multitude of diseases may increase the pulmonary artery pressure and result in right ventricular dysfunction. The treatments of pulmonary hypertension are as varied as its causes. The past decade has realized remarkable growth in knowledge of the mechanisms of pulmonary arterial hypertension and, concurrently, therapies for this once uniformly fatal disease. In addition to continuous intravenous epoprostenol, subcutaneous treprostinil and oral bosentan are now FDA approved for the treatment of pulmonary arterial hypertension. Other forms of pulmonary hypertension, such as pulmonary venous hypertension, pulmonary hypertension related to diseases of the respiratory system, and thromboembolic pulmonary hypertension will be discussed.

Cardiotonic Agents↗

Long-term outcome of right ventricular outflow tract reconstruction using a handmade tri-leaflet conduit.

OBJECTIVE: Since 1985, we have implanted handmade tri-leaflet conduits made of heterologous pericardium or expanded polytetrafluoroethylene (ePTFE), as an alternative to homograft for right ventricular outflow tract reconstruction. This report assesses the long-term outcome of these prostheses. METHODS: From 1985 to 2003, 216 handmade tri-leaflet conduits were implanted in 191 patients. Forty-two patients had previous conduit repairs. The mean age at operation was 8.1+/-7.7 years (range: 15 days-44 years). The underlying diagnoses were pulmonary atresia with ventricular septal defect in 83 patients, atrioventricular discordance in 36, transposition of the great arteries in 26, double outlet right ventricle in 14, and truncus arteriosus in 17. Whole heterologous pericardial tri-leaflet conduits were implanted in 169 patients, in the early series (porcine: 85; equine: 58; bovine: 26); bovine pericardial conduits containing ePTFE leaflets were implanted in 26 patients since 1996; whole ePTFE tri-leaflet rolls were employed in the most recent 21 patients. The conduit size was 21.1+/-3.1mm (range: 12-27 mm), 147.4+/-21.4% (range: 82.6-202.6%) of the anticipated diameter of the pulmonary valve. Follow-up was complete. RESULTS: There were 28 early deaths and 24 late deaths. The indication for conduit replacement was a peak instantaneous pressure gradient of greater than 50 mmHg. Sixty-five conduits required reoperation for conduit obstruction at 8.6+/-3.3 years after implantation. The freedom from reoperation at 5, 10, and 15 years was 93.9+/-1.9, 61.4+/-4.5, and 35.5+/-5.6%, respectively. Patients with smaller conduit size and young age at operation were predisposed to reoperation. None of the 47 ePTFE tri-leaflet conduits developed significant obstruction. The freedom from important pulmonary valve regurgitation (PR) as assessed by echocardiography was 68.3+/-3.7% at 5 years, 33.0+/-4.5% at 10 years, and 21.6+/-4.9% at 15 years. No patient required reoperation due to PR or right ventricular dysfunction. CONCLUSIONS: Handmade tri-leaflet conduits provide a reliable alternative for RVOT reconstruction in children, yielding as good a long-term outcome as do homografts. Longer follow-up is needed to determine how well ePTFE leaflets will fare.

Adolescent↗

Pulmonary embolism and cardiac enzymes.

BACKGROUND: Pulmonary embolism (PE) is often associated with chest pain, electrocardiographic changes, and right ventricular (RV) dysfunction on echocardiogram. There have been reports of elevated troponin levels with PE. RV dysfunction and elevated troponin levels have prognostic implications in acute PE. The purpose of this retrospective analysis was to determine whether PE was associated with elevated cardiac enzymes and whether there was any difference among patients who presented with or without chest pain. METHODS: Records of 93 consecutive patients with high-probability ventilation/perfusion lung scan results for PE were analyzed for the presence or absence of chest pain on presentation, abnormalities in cardiac enzymes, and evidence of RV dysfunction on echocardiogram. RESULTS: A total of 56 of 93 patients had cardiac enzymes evaluated; 24 of these 56 patients had chest pains, and 32 did not. Only 1 patient of the 56 had abnormal cardiac enzymes. This patient had a known history of coronary artery disease (CAD) and had experienced an acute anterior myocardial infarction. Echocardiograms were performed in 36 of 93 patients. Evidence of RV dysfunction on echocardiograms was found in 22 of these patients. No significant relationship was found between RV dysfunction and chest pains (P > .10). CONCLUSION: We found no significant relationship between high-probability ventilation/perfusion scan results and abnormalities in cardiac enzymes irrespective of the presence or absence of chest pain. Patients with a history of CAD or RV dysfunction did not have a higher incidence of chest pain when compared with those with no known history of CAD or RV dysfunction.

Adult↗