Search

To define current role of the Ross operation in young patients, the outcome in those followed longer than 20 years were assessed. Between 1994 and 2020, 81 consecutive patients, 70 of 11 male and/or female, mean age 27 years underwent Ross procedure, accruing 20 years of follow-up or longer. Sixty-four had bicuspid valve (79%) and 54 (67%) aortic insufficiency, while 15 (19%) had undergone prior operations. Surgery consisted in root replacement in 53 patients, cylinder inclusion in 20 and sub-coronary graft in 8. There were 7 late deaths in 80 hospital survivors (median follow-up 21 years, IQR 20-23), with 88% ± 5% survival at 25 years. Thirty-four patients required left, 6 left and/or right and 1 right heart valve reoperation, on average 13 years after Ross procedure. Reoperation was valve-sparing in 18 (45%) patients and valve and/or root replacement in 22. Ten (24%) reoperated patients required a second reoperation 18 years after Ross procedure. Freedom from autograft reoperation was 46% ± 6%, while from autograft valve replacement was 60% ± 7%, thanks to autograft valve-sparing. Freedom from isolated right valve reoperation was 98% ± 4%. No mortality was associated with any of the 51 reoperations. Root technique was associated with reoperation (P = 0.024). Age at follow-up was 50 years (IQR 36-60), with 70 (96%) patients in NYHA class I and 6 (55%) women carrying out pregnancies. Young patients undergoing the Ross procedure enjoy unprecedented survival well into the third decade of follow-up, even when faced with reoperation. At 25 years risk of autograft reoperation is consistent, while negligible for homograft. Technical improvements at operation and valve-sparing at reoperation may prolong autograft valve durability., (Copyright © 2022 Elsevier Inc. All rights reserved.)

(1) Objective: To evaluate: (i) the associations of age and disease severity with anthropometric indices and weight status, (ii) the difference in the frequency of sports activity among different levels of disease severity in paediatric patients with congenital heart disease (CHD). (2) Methods: Clinical data of Caucasian children (aged 2-18 years) diagnosed with CHD (2005-2018) were retrospectively collected from the electronic register of outpatient visits. Of the 475 children with CHD, 368 children and their 1690 complete anthropometric measurements were eligible for inclusion in our analysis. (3) Results: Significant increase with age was observed for weight z-score [beta (95%CI): 0.03 (0.02, 0.05) for one-unit of age] and BMI z-score [0.06 (0.03, 0.08)] but not for height z-score. The probability of being underweight and overweight/obese increased and decreased with disease severity, respectively. The obesity probability of patients with mild CHD (0.06 [95%CI: 0.03, 0.08]) was not statistically distinguishable from that of patients with moderate CHD (0.03 [95%CI: 0.02, 0.05]), whereas it was lower in patients with severe CHD (0.004 [95%CI: 0.0, 0.009]). No obese patients with a univentricular heart defect were observed. Days spent in sport activities were equal to 1.9 [95%CI: 1.6, 2.2] days/week, 1.9 [1.5, 2.2], 1.4 [1.1, 1.7] and 0.7 [0.1, 1.3] in patients with mild, moderate, severe and univentricular CHD, respectively. (4) Conclusions: The risk of being overweight and obese should not be underestimated in paediatric patients diagnosed with CHD, especially in children with mild or moderate heart defects. It could be prevented or reduced by promoting a healthy lifestyle.

Aim: Bicuspid aortic valve (BAV) is the most common congenital heart defect, with considerable risk of morbidity and mortality. The purpose of the study was to analyze clinical and echocardiographic presentation of BAV in a large-volume tertiary Italian center and to test their interaction with full age span, sex, and first diagnosis versus second referral., Methods: Consecutive patients of all ages diagnosed with BAV at our center from January 1988 to December 2012 were retrospectively included. Exclusion criteria were as follows: associated complex congenital cardiac disease, systemic syndrome, and previous cardiac surgery., Results: Eligible patients were 790, divided by age quartiles. Seventy-two percent of patients had any grade BAV dysfunction. Aortic valve stenosis was more frequent in the first (24%) and fourth (24%) quartiles. This corresponds to a double-peak stenosis severity curve, being more severe at a very young age and in the elderly. Aortic valve regurgitation was more prevalent in each quartile than stenosis, with a prevalence of 72% in the second quartile and 77% in the third quartile. This corresponds to a single-peak regurgitation severity curve, being more severe in the fourth and fifth decades of life. Patients with previously diagnosed BAV had more significant valve dysfunction in comparison to patients with first diagnosis of BAV, either stenosis (15% vs. 21%, P = 0.024) or regurgitation (58% vs. 68%, P = 0.006)., Conclusion: The dominant BAV dysfunction in this large Northern Italian community is regurgitation, with higher severity of disease in the fourth and fifth decades of life., Competing Interests: There are no conflicts of interest., (Copyright: © 2021 Journal of Cardiovascular Echography.)

Mucopolysaccharidoses (MPS) represent a group of rare lysosomal storage disorders, with a heterogeneous clinical presentation in terms of inheritance (autosomal and X-linked recessive), age of onset (infants, children, and adults), systemic and cardiac manifestations (mild to severe disease forms). Evidence-based recommendations on the diagnosis and management of cardiovascular disease in MPS are scarce. GICEM (Gruppo Italiano Cardiologi Esperti Malattie Metaboliche) is a group of cardiologists, cardiac surgeons and pediatricians with a specific expertise in metabolic diseases including MPS. In this paper, we report our experience and recommendations on the diagnosis and management of cardiovascular aspects in MPS, with a tailored approach based on current evidence, and taking into account MPS phenotype (particularly, I, II, IVa, VI), age at presentation, and severity of systemic and cardiac manifestations.

Objective: To assess accuracy and repeatability of a modified echocardiographic approach to quantify superior vena cava (SVC) flow volume that uses a short-axis view to directly measure SVC area and a suprasternal view to measure flow velocity, both at the level of the right pulmonary artery., Setting: Three tertiary-level neonatal intensive care units., Design: This was a multicentre, prospective, observational study. Accuracy of the traditional and modified approach was first assessed by comparing echo measurements according to both techniques with Phase contrast MRI (PCMRI) assessments, in a cohort of 10 neonates. In a second cohort of 40 neonates, intraobserver scan-rescan repeatability and interobserver analysis-reanalysis repeatability were assessed by repeated SVC flow echo measurements, according to both techniques., Results: The traditional echocardiographic approach to assessment of SVC flow had a moderate agreement with PCMRI (r 2 0.259), a scan-rescan intraobserver repeatability index (RI) of 37% (limits of agreement (LOA) -47/+51 mL/kg/min) and an interobserver analysis-reanalysis RI of 31% (LOA -38/+40 mL/kg/min). The modified approach showed a stronger agreement with PCMRI (r 2 0.775), an improved intraobserver scan-rescan repeatability (RI 22%, LOA -24/+18 mL/kg/min) and improved interobserver analysis-reanalysis repeatability (RI 18%, LOA -18/+20 mL/kg/min)., Conclusions: Echocardiographic assessment of SVC flow volume by tracing area from a short-axis view and measuring velocity-time integral from a suprasternal view offered an improvement in accuracy and repeatability, building on the traditional approach previously described., (Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/.)

: We report the case of a 56-year-old male patient affected by a severe form of Ebstein's anomaly (type C of Carpentier classification) with secundum atrial septal defect, who presented to the emergency department with impaired consciousness, seizures, and trismus. The brain computed tomography scan showed evidence of a mass located in the frontal lobe, confirmed by brain MRI consistent with brain abscess. Both echocardiography and cardiac MRI showed no evidence of valvular vegetation. This case shows how the combination of increased atrial pressure and bidirectional shunt through atrial septal defect may lead to paradoxical embolization.

Background: Pulmonary hypertension (PH) is often found in cystic fibrosis (CF) patients affected by end-stage lung disease but its impact on outcome remains unclear. Pulmonary arterial compliance (PAC) is an important determinant of right ventricle (RV) workload and it is a strong predictor of survival in other forms of PH. The aim of this study is to investigate whether PAC is a predictor of long-term prognosis in a population of CF patients affected by advanced lung disease., Methods: Between 2000 and 2014, 178 patients with CF have been evaluated for lung transplantation in our CF Center. Right heart catheterization (RHC) and follow up data were retrievable and analyzed in 141 of them. PAC was defined as the ratio between stroke volume (SV) and pulse pressure (PP) at heart catheterization. The association of PAC with survival was tested at 4 years and compared to other hemodynamic parameters., Results: PH prevalence was 56.4%. Most patients had mild elevation of pulmonary artery pressure (PAP). No difference in mortality was observed in patients with PH compared to patients with normal PAP (HR 0.95: 95% CI 0.49-1.89, p=0.89). At receiver operating characteristic curve (ROC) analysis, the optimal prognostic cut-off point of PAC was 1.95 ml/mmHg. An impaired PAC (≤1.95 ml/mmHg) was a strong independent predictor of long-term mortality (HR 3.44: 95% CI 1.51-7.85: p=0.003)., Conclusions: Impaired PAC is associated with poor prognosis in CF patients awaiting lung transplantation. Other traditional hemodynamic parameters add no prognostic information., (Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.)

Pre-eclampsia complicates approximately 6-8% of all pregnancies. Epidemiologic studies have demonstrated a relationship between pre-eclampsia and cardiac morbidity and mortality later in life, but the effect of pre-eclampsia on electrical cardiac activity during the acute phase has not yet been understood. The aim of this study was to investigate ECG alterations during pre-eclampsia. Prepartum ECGs of 76 consecutive pre-eclamptic women were compared with those of 76 healthy pregnant women. All of the routine ECG parameters were considered, and ventricular repolarization was assessed by QT interval and QT dispersion (QTd). Pregnancies complicated by pre-eclampsia showed a significant alteration of ventricular repolarization compared with the control group. Among ECG parameters, QT and QTc intervals and QTd were more prolonged in pre-eclamptic women. Multivariate analysis also showed that pre-eclampsia was the only independent determinant of QTd. In conclusion, pre-eclampsia has a significant effect on ventricular repolarization. This alteration could, in part, explain the increased cardiovascular risk of women with a history of pre-eclampsia. Further studies are necessary to confirm the relationship between ventricular repolarization abnormalities and increased cardiovascular risk later in life.

We report the case of a large congenital rhabdomyoma of the interventricular septum diagnosed prenatally. The foetus was strictly monitored with ultrasound throughout the gestation period showing that the mass had increased in size until delivery. Despite the size of the mass, which appeared to occupy the right ventricle, the baby presented no symptoms both in utero and after birth. Serial echocardiography was used to document the regression of the mass in childhood.

Aims: Cardiac resynchronization therapy (CRT) is currently considered an important breakthrough in the treatment of selected patients with refractory heart failure. However, long-term predictors of mortality, morbidity and time to recovery of ventricular function for those patients who respond positively to CRT remain poorly investigated., Methods: This is a retrospective follow-up study involving one hospital. Between August 2004 and October 2008, 211 consecutive patients with refractory heart failure received a CRT device in the Cardiology Division of Ospedale Civile Maggiore in Verona. The clinical characteristics studied were age, sex, heart rhythm, left ventricular end-systolic volume/body surface area (LVESV/BSA), left ventricular ejection fraction, QRS duration, type of bundle-branch block, cause, New York Heart Failure Association functional class, pharmacological therapy and lead position. The objective of this study was to evaluate the effect of several baseline characteristics on long-term prognosis in heart failure patients treated with CRT., Results: Nonischemic cause, left bundle-branch block and a basal LVESV/BSA of 106 ml/m or less were the only independent predictors of a positive response to CRT (P < 0.005). Additionally, a reduction in LVESV/BSA after CRT was associated both with increased survival and reduced rehospitalization for heart failure (P < 0.005)., Conclusion: A better selection of patients on the basis of cause, type of bundle-branch block and basal LVESV/BSA can increase the number of patients that would benefit from CRT.

Double-outlet left ventricle is an exceedingly rare congenital heart defect. Its prenatal detection and precise anatomical definition are challenging for a variety of reasons and have never been previously reported. Here described are 2 cases of prenatally diagnosed double-outlet left ventricle. The technical limitations of prenatal diagnosis and its implications for the surgical management of patients affected by such a rare condition are discussed.

The atrial switch (Mustard, Senning procedures) was one of the treatments of choice for repair of transposition of the great arteries from the early 1960s to the mid-1980s. A significant proportion of patients with atrial switch develops systemic (right) ventricular failure. A series of surgical therapeutic options exists to manage cardiac failure in this setting, and, more recently proposed, cardiac resynchronization therapy. We describe case report of a 30-year-old woman with congenital heart disease (CHD) and previous Mustard procedure who underwent upgrading from single chamber to dual-chamber pacemaker. The narrower native QRS did not correlate with a better synchrony status nor with a better cardiac output. Functional evaluation confirmed a better performance in DDD mode with short atrioventricular delay and broad QRS. Some echocardiographic and electrocardiographic parameters, such as ejection fraction and QRS duration, well established in adults' heart for selection of candidates to cardiac resynchronization therapy, are much less studied in CHD. Postoperative CHD may provide unique patterns of asynchrony with poorly predictable hemodynamic outcome., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Aortic intramural hematoma is a life-threatening thoracic aortic pathology. In this report we describe a case of fissuration of an aortic intramural hematoma with atypical clinical presentation, which occurred in an aircraft pilot. The patient was admitted to our emergency room with transient chest pain developed during a flight landing, followed only by persistent abdominal pain. The ECG and cardiac enzymes were normal. A portable two-dimensional transthoracic echocardiogram showed aortic root dilation and pericardial effusion. Transesophageal echocardiography showed aortic intramural hematoma with fissuration into the pericardial space. The angio-computed tomography confirmed the diagnosis. Two hours after admission the patient, with signs of cardiac tamponade, underwent Bentall surgical intervention without complications.

This report describes an unusual case with tortuosity of the great vessels in a neonate who presented at birth with cyanosis. The diagnosis was made with magnetic resonance imaging (MRI), then confirmed by genetic analysis.

Background: Arterial tortuosity syndrome (ATS) (OMIM #208050) is a rare autosomal recessive connective tissue disorder characterized by tortuosity and elongation of the large and medium-sized arteries, propensity to aneurysms formation, vascular dissection, and pulmonary arteries stenosis. ATS is caused by mutations in SLC2A10 gene, encoding for the facilitative glucose transporter 10 (GLUT10). So far, 17 SLC2A10 mutations have been reported in 32 families, two of which were Italian with a total of five patients. Here we present the clinical and molecular characterization of two novel Italian paediatric ATS patients., Methods: The exons and intronic flanking regions of SLC2A10 gene were amplified and direct sequencing was performed., Results: In both patients, the involvement of major- and medium-sized arteries was characteristic; the nonvascular connective tissue manifestations were mild and not pathognomic of the disorder. Both patients, born from non-consanguineous parents, were heterozygous for two different SLC2A10 mutations, three of which were recurrent and one was novel (p.Arg231Trp). This mutation is localized at the endofacial loop between the transmembrane domains 6 and 7 of GLUT10., Conclusion: Two novel ATS patients were characterized at clinical and molecular level. Overall, four ATS unrelated families are known in Italy so far. Though ATS clinical delineation improved in the last years, further works in the comprehension of disease presentation and complications onset, particularly in paediatric age, and on ATS molecular basis are needed to add new insights for diagnosis and prevention strategies for related complications.

Diagnosis of arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is often challenging due to differing clinical presentations and unpredictable progression of the disease. We report a case of ARVD/C that presented as cardiac arrest in an 18-year-old male while playing soccer. The electrocardiographic features after resuscitation were typical of anterior ST-segment elevation acute myocardial infarction, and the patient was initially managed accordingly. Importantly, an urgent coronary angiogram revealed completely normal coronary arteries. ARVD/C was first suspected following an echocardiogram, and was later confirmed by cardiac magnetic resonance. One month before the event, the patient had been evaluated for ventricular extrasystoles and an abnormal resting electrocardiogram, however ARVD/C was ruled out because of the presence of only two minor diagnostic criteria (T-wave inversion in anterior precordial leads in the absence of right bundle branch block and more than 1000 ventricular extrasystoles during 24-h Holter monitoring). In consequence, physical activity was not forbidden. In conclusion, this case report enforces the need for a strict prohibition of physical activity and serial evaluation of individuals with only minor diagnostic criteria for ARVD/C, for lacking sensibility of Task Force diagnostic criteria.

Neonatal fungal valve endocarditis is an uncommon and highly lethal disease. The ideal management strategy is still controversial. Current options include antifungal chemotherapy and surgical intervention, the latter being often limited by risks inherent with valve operations in low body weight infants. We present a case of a premature infant with multiple Candida tricuspid valve mycetomas. Eradication of infection was achieved by combined liposomal amphotericin therapy and complex tricuspid valve repair. Indications, technical aspects, and outcome of treatment in infants are reviewed.

Aim: An early diagnosis of congenital heart disease (CHD) is necessary for the obstetrical management., Methods: One thousand five hundred and fifty-six pregnant women underwent a fetal 2-D echocardiography from 1991 to 2002. We assessed patients who had the most common risk factor (RF): family history of CHD, diabetes mellitus, teratogen drugs, polyhydramnios, abnormal fetal growth, fetal arrhythmia, maternal age over 40 years, maternal autoimmune disease, maternal disease contracted during pregnancy, oligohydramnios, only umbilical artery, uncorrected visualization in the first level 2-D fetal echocardiography., Results: In 110 morphological anomalies found 54 were complex. The malformations are not equally distributed among the different RF. The most common RF is the family history of CHD where 24 patients showed complex malformations. Another high percentage of complex malformations was found in patients with no apparent presence of RF: the diagnosis was done after an uncorrected cardiac visualization in the first level 2-D echocardiography. In the minor cardiomyopathies we did not find the prevalence of any RF. After diagnosis of complex malformations we had 15% of termination of pregnancies, 37% of the newborns are alive and in good health., Conclusions: The first RF is the family history of CHD, but a correct first level fetal 2-D echocardiography is necessary because a very high percentage of complex malformations is seen in infant whose mother did not have any RF. Ninety percent of cardiac malformations where seen in a 4 chamber view, and it is of the utmost importance also for a correct diagnosis of minor cardiomyopathies where we did not find a predominant RF.

Background: The Ross operation is an alternative to mechanical aortic valve replacement in the young. Early dilatation of the pulmonary autograft root exposed to the systemic circulation has been reported. To define the prevalence of, risk factors for, and consequences of late autograft dilatation, outcome in all consecutive patients operated since May 1994 was reviewed., Methods and Results: Ninety one patients, 77 males and 14 females, with at least 1 year of follow-up underwent cross-sectional clinical and echocardiographic examination. Age at operation was 27+/-10 years (range 6 to 49), and the indication was aortic regurgitation in 54 (59%) patients and bicuspid valve was present in 62 (68%). End-points of the study were freedom from autograft dilatation (root diameter >4 cm or 0.21 cm/m2), from (moderate) autograft regurgitation and from reoperation. Follow-up (4.0+/-1.9, range 1 to 8 years) autograft root diameters were anulus, 29+/-4 mm (18-39); sinus of Valsalva, 38+/-7 mm (24-53); sinotubular junction, 37+/-6 mm (23-54); and ascending aorta, 37+/-5 mm (27-54). Late autograft dilatation was identified in 31 (34%) patients and regurgitation in 13 (14%), 7 of whom had autograft dilatation. At 7 years, freedom from dilatation was 42+/-8%, freedom from regurgitation was 75+/-8%, and freedom from reoperation was 85+/-10%. Cox proportional hazard analysis identified younger age (P=0.05), preoperative sinus of Valsalva (P=0.02), root replacement technique (P=0.03), and absence of pericardial buttressing (P=0.04) as predictive of autograft dilatation, whereas female sex (P=0.002), follow-up sinus of Valsalva (P=0.003), and sinotubular junction diameter (P=0.02) as predictive of autograft regurgitation., Conclusions: Autograft dilatation is common late after the Ross procedure, particularly in younger patients, in those with preoperative aortic aneurysm, and those having root replacement without support of anulus and sinotubular junction. Bicuspid aortic valve is not a risk factor. Significant autograft valve dysfunction affects a minority of patients, but it is more prevalent in those with autograft dilatation.

Introduction: Both surgical techniques for correction of congenital heart diseases (CHD) and intraoperatory neurologic protection improved during the last 20 years. Nevertheless cardiac surgery is still a risk for neurologic morbidity., Methods and Patients: Analysis of the postoperative neurologic status of infants younger than 6 months who underwent cardiac surgery from January 1998 to December 1999. We reviewed the EEG tracings, cranial ultrasound reports (CUS) and CT scans of 48 patients. Diagnoses were: ventricular septal defect = 15, Fallot (TOF) = 9, patent ductus arteriosus (PDA) = 5, coarctation of aorta = 4, atrio-ventricular septal defect = 4, transposition of great arteries (TGA) = 3, hypoplastic left heart syndrome = 2, pulmonary atresia = 2, total anomalous pulmonary veins drainage = 2, double outlet right ventricle = 1, cor triatriatum = 1. Mean age (range) at intervention was 54 days (2-150), 44 infants (91.7%) survived at follow-up: 23 EEG, 22 CUS and 2 CT were performed in the recent postoperative. Among survivors 5/44 had neurologic complications. EEG was altered in 4: two of them (1 TOF, 1 TGA) had pathologic CUS and CT as well (ischemic pattern in the former, atrophy in the latter). Finally a preterm newborn with PDA had mild abnormalities at CUS. After a mean follow-up of 16 +/- 6 months 3/5 patients had mild-to-moderate psychomotor delay and 2 recovered., Conclusions: According to our preliminary data the prevalence of neurologic complications in infants who undergo cardiac surgery seems to be low. The pathological findings of the recent postoperative seem to recover up to normalization in some cases at mid-term follow-up. As expected, permanent complications effect more often complex CHD. Further follow-up studies to school age will be mandatory to check the very final results of cardiac surgery performed during early infancy.

Objectives: The objective was to analyze the accuracy and diagnostic value of the estimated regurgitant volume of mitral regurgitation using 1) left atrial volume variation during ventricular systole (left atrial filling volume) and 2) the percent of systolic pulmonary vein velocity integral compared with its total., Background: Left atrial filling volume (LAfill), which represents the atrial volume variation during ventricular systole, has been used for the assessment of mitral regurgitation severity. A good correlation with invasive semiquantitative evaluation was found, but with an unacceptable overlapping among grades. The reason could be the absence of information concerning the contribution of blood entering into the left atrium from the pulmonary veins., Methods: Doppler regurgitant volume (Dpl-RVol) (mitral stroke volume - aortic stroke volume) was measured in 30 patients with varying degrees and etiological causes of mitral regurgitation. In each patient atrial volumes were measured from the apical view, using the biplane area-length method. The systolic time-velocity integral of pulmonary vein flow was expressed as a percentage of the total (systolic-diastolic) time-velocity integral (PVs%). These parameters were used in this group of patients to obtain an equation whose reliability in estimating Dpl-RVol was tested in a second group of patients., Results: In the initial study group, with linear regression analysis the following parameters correlated with Dpl-RVol: end-systolic left atrial volume (R2=0.37, p=0.0004); LAfill (R2=0.45, p < 0.0001); PVs% (R2=0.56, p < 0.0001). In multiple regression analysis the combination of LAfill and the percent of the systolic pulmonary vein velocity integral (PVs%) provided a more accurate estimate of regurgitant volume (R2=0.88; SEE 10.6; p < 0.0001; Dpl-RV=6.18 + (1.01 x LAfill) - (0.783 x PVs%). The equation was subsequently tested in 54 additional patients with mitral regurgitation with a mean Dpl-RVol 27+/-37 ml. Estimated regurgitant volume and Dpl-RVol correlated well with each other (R2=0.90; SEE 12.1; p < 0.0001). In the test population, the equation was 100% sensitive and 98% specific in detecting a regurgitant volume higher than 55 ml., Conclusions: Left atrial filling volume and pulmonary vein flow give a reliable estimate of regurgitant volume in mitral regurgitation.

Objective: The purpose of this study was to evaluate infarction-related changes in the infarcted and the non-infarcted myocardium using a baseline assessment of ventricular function obtained prior to the infarction., Background: Experimental studies have shown that both infarcted and non-infarcted myocardium contribute to the process of left ventricular dilatation soon after the infarction, but no data exist on the effect that the infarct has on the pre-infarct ventricular morphology in humans., Methods and Results: 10 patients, out of 721 admitted to our coronary care unit with a first acute myocardial infarction over a 3-year period, had had an echocardiographic examination performed before (354 +/- 407 days) and after (10 +/- 9 days) the infarction which were adequate for quantitative evaluation. Ventricular volume (Simpson) and regional wall motion (Centerline method) were evaluated by biplane apical sections and the endocardial length of the infarct and the non-infarct segments, imaged in a cross-sectional view at the papillary muscle level, were measured. After the infarction end-diastolic and end-systolic ventricular volume increased (P = 0.0003 and P < 0.0001, respectively); diastolic and systolic infarct segment length increased (P = 0.011 and P = 0.0008, respectively), while non-infarct segment had only diastolic lengthening (P = 0.019), without systolic changes. The ejection fraction decreased after the infarction (P < 0.0001), in inverse relation to infarct size and in direct relation to diastolic non-infarct segment lengthening. In the five patients in whom there was a significant diastolic lengthening of non-infarct segment (larger than mean +/- 2 S.D. of the interobserver variability) the decrease in ejection fraction was less than in the patients without significant lengthening of this segment (P = 0.017), despite a similar echocardiographic infarct size index., Conclusion: Ventricular enlargement early after myocardial infarction is due to both infarct expansion and lengthening of non-infarct segment. However, while systolic stretching of the infarct segment is a deleterious process that accounts for the increase in end-systolic volume, diastolic non-infarct segment lengthening is the expression of a functional compensatory mechanism that counteracts the reduction of the ventricular pump function secondary to the infarction.

Prostaglandin E1 improves hemodynamics in patients with severe dilated cardiomyopathy and pulmonary hypertension through it's reducing action on pulmonary resistances. However, few data are available to indicate whether these beneficial effects on right heart hemodynamics translate into any improvement of the altered left ventricular filling pattern that characterizes this condition. We studied 12 patients with dilated cardiomyopathy during preoperative evaluation for cardiac transplantation before and after prostaglandin E1, 30-50 ng/kg/min i.v. Patients underwent catheterization of the right heart and left ventricle by Swan-Ganz catheter, giving simultaneous assessment of pressure by micromanometer and of volume derived from two-dimensional echo-guided Doppler mitral flow velocity, where volume equals mitral velocity integral x valvular area. Prostaglandin E1 induced a significant reduction in mean pulmonary (from 38 to 30 mm Hg; p = 0.0001) and aortic (from 79 to 75 mm Hg, p = 0.05) pressures but no change in heart rate or tau. Peak A wave increased from 28 to 33 cm/s (p = 0.02), along with a reduction in end-diastolic pressure from 29 to 26 mm Hg (p < 0.04), whereas peak E wave did not change. E/A ratio decreased slightly (from 2.5 to 2.1; p < 0.0007) but did not reverse. Systolic volumes decreased (from 231 to 212 ml; p < 0.05), and cardiac index increased from 2.1 to 2.6 L/min/m2 (p = 0.0002) because of a reduction in pulmonary and systemic vascular resistances. The diastolic pressure-volume relation shifted downward along the same curve. Prostaglandin E1 infusion in patients with severe dilated cardiomyopathy and pulmonary hypertension reduces pulmonary and systemic resistances without affecting heart rate, relaxation, or passive diastolic left ventricular properties. Systolic right and left ventricular unloading increases cardiac index, facilitating ventricular emptying. E/A ratio does not reverse, although it decreases slightly, with mechanisms, however, that appear independent of any direct effect of the drug on the ventricular diastolic properties.

The usefulness of transesophageal atrial pacing combined with 2-dimensional echocardiography (echo-pacing) in predicting the presence and site of jeopardized myocardium, defined as areas of myocardium perfused by a vessel with a stenosis > or = 75% or by a collateral circulation if the supplying vessel was occluded, was evaluated in 31 patients with uncomplicated acute myocardial infarction who underwent coronary angiography. All 5 patients without jeopardized myocardium had a negative test, whereas 24 of 26 with jeopardized muscle had a positive test (sensitivity 92%; specificity 100%). To identify the site of jeopardized myocardium, tests that were positive for development of new asynergies were analyzed further, distinguishing those positive in the infarct or remote zone. Seven of 8 patients with new asynergies in the remote zone had areas of jeopardized myocardium outside the territory of distribution of the infarct-related vessel, whereas only 2 of 12 with new asynergies in the infarct zone had areas of jeopardized myocardium outside that territory (p < 0.01), correctly predicting the site of jeopardized myocardium in 17 of 20 cases. In conclusion, echo-pacing is useful for detecting the presence and site of jeopardized myocardium after an acute myocardial infarction.

Reperfusion reduces left ventricular dilatation in patients with acute myocardial infarction, but it is unclear to what extent this is a primary effect or only a consequence of the limiting effect of reperfusion on infarct size. To address this issue, 56 consecutive patients were examined by means of two-dimensional echocardiography on day 1, on day 3, before discharge, and at 6 months after an acute myocardial infarction. From this population two groups of 12 patients each, perfectly matched for site of myocardial infarction, extent of ventricular asynergy at two-dimensional echocardiography (akinesis + dyskinesis), and clinical characteristics were identified according to the creatine kinase (CK) time to peak, which was regarded as a marker of spontaneous or induced reperfusion: (1) CK time to peak of 12 hours or less (reperfused patients, n = 12), and (2) CK time to peak of more than 12 hours (nonreperfused patients, n = 12). In these two groups of patients end-diastolic and end-systolic left ventricular volumes and endocardial lengths of asynergic and normal ventricular segments, imaged in a cross-sectional view at the level of the papillary muscles, were then computed. At the first examination end-diastolic volume, end-systolic volume, and endocardial segment lengths of normal and asynergic segments were similar in the two groups of patients. Patients with late CK time to peak, however, showed a progressive increase in left ventricular systolic volumes and in asynergic endocardial segment lengths between the first and third (predischarge) examinations (p < 0.05 for both), with no change in systolic length of the normal myocardium. The left ventricular end-systolic volume and the asynergic endocardial segment length of patients with early CK time to peak, however, did not increase during hospitalization. The increment in end-systolic volume and in systolic infarct segment length from the first to the third examinations was higher in nonreperfused patients (p = 0.018 and p = 0.04, respectively). Changes similar to those detected in systole were found for diastolic volume and diastolic infarcted and noninfarcted segment length in both groups, but they did not reach statistical significance. After 6 months, an increases in volume and endocardial length were found in both groups of patients. Relative to the first examination, however, the increase in systolic volume and in asynergic systolic endocardial lengths remained greater for nonreperfused patients (p = 0.077 and p = 0.01, respectively).(ABSTRACT TRUNCATED AT 400 WORDS)

The importance of infarct expansion in determining global ventricular remodelling and prognosis after myocardial infarction is well known, whereas how infarct expansion affects left ventricular filling dynamics is not defined. To address this issue two-dimensional and Doppler echocardiography was performed in 28 consecutive patients admitted to our Coronary Care Unit for a first acute transmural myocardial infarction 1) within 24 hours of symptoms' onset and 2) at predischarge. A semiquantitative echocardiographic infarct size index was computed, while the infarct and non-infarct segment length was measured in a short-axis papillary muscle section. Peak velocity of early (E) and late (A) transmitral Doppler curves were also measured. An increment in infarct segment length > or = 1.2 cm between baseline and predischarge examination was chosen as target to divide patients with (N = 8) and without (N = 20) infarct expansion. Patients with expansion had a higher echocardiographic infarct size index (3.5 +/- 1.4 versus 2.3 +/- 0.6 segments, p < 0.0001) and a higher CK-MB infarct size (336 +/- 235 versus 129 +/- 87 UI, p = 0.002), while ejection fraction was lower (36 +/- 8% versus 48 +/- 7%, p < 0.001). A linear correlation was found between the increment in infarct segment length and in left ventricular volume between the baseline and the predischarge examination (r = 0.58, p < 0.01). Doppler parameters were not different at baseline examination between patients with and without expansion.(ABSTRACT TRUNCATED AT 250 WORDS)

In order to investigate the effects of increasing degrees of left ventricular filling impairment on left atrial function, in 9 A-fillers (E/A ratio less than 1, E wave deceleration time greater than 170 ms) and 9 E-fillers (E/A ratio greater than 1, E wave deceleration time less than 150 ms) we constructed the left ventricular and the left atrial volume curves according to a previously validated Doppler 2-dimensional echo method which combines mitral and pulmonary venous flow. Eight normals served as control. The left atrial reservoir (defined as maximum-minimum atrial volume), pump (defined by the volume of blood that enters the left ventricle with the atrial contraction) and conduit functions (defined as left ventricular filling volume--the reservoir and the pump volume) expressed as % of the left ventricular filling volumes, varied significantly between normals (37 +/- 9%, 25 +/- 3%, 37 +/- 11%), A-fillers (48 +/- 9% p less than 0.05, 39 +/- 5% p less than 0.05, 14 +/- 10% p less than 0.001) and E-fillers (27 +/- 6% p less than 0.05, 19 +/- 7% p less than 0.05, 54 +/- 10% p less than 0.01). Also maximum left ventricular and left atrial volumes differed significantly (normals 165 +/- 31 ml, 76 +/- 20 ml; A-fillers 174 +/- 33 ml, 100 +/- 20 ml p less than 0.05; E-fillers 322 +/- 34 ml p less than 0.001, 136 +/- 41 ml p less than 0.01).(ABSTRACT TRUNCATED AT 250 WORDS)

The contrast agent SHU-454 was intravenously injected in 103 patients during echocardiography: 37 mL/patient +/- 7 ml/patient. The quality of the contrast effect was optimal in 13, good in 51, sufficient in 30, and poor in 9 patients; reproducibility was optimal in 38, good in 46, sufficient in 17, and poor in 2 patients. Taste sensation, arm discomfort, or atypical chest pain occurred in 5 patients, premature ventricular contractions in 3. An isolated anginal attack occurred in 1 patient with frequent episodes at rest. A slight reduction in hematologic indices was attributed to hemodilution. Thus, SHU-454 produces a good and reproducible contrast effect with tolerable side effects.

Seventeen of the centres participating to the G.I.S.S.I. trial performed also, before discharge from the Hospital, an echocardiographic examination of patients (pts) included in the study. 561 pts were included, 280 assigned to the streptokinase (SK) treatment, and 281 to the control (CT) group. The echocardiographic asynergic area score index was lower in the SK pts than in the CT group (p less than 0.01). The difference was more evident in pts treated within 6 hours from the onset of symptoms (p less than 0.005), in pts without previous infarct (p less than 0.005), and in pts aged over 65 (p less than 0.005). The end diastolic (EDV) and the end-systolic (ESV) volumes were lower in SK pts (p less than 0.01 and p less than 0.025 respectively) than in the CT group; the ejection fraction (EF) did not differ. The reduction of EDV and ESV was more evident in pts treated within 6 hours, in pts without previous infarct, in pts aged over 65, and in anterior infarcts. At the 6-month follow-up examination, in SK pts the asynergic area score index, the EDV, the ESV and the EF were unmodified; in CT pts, on the contrary, the EDV and the ESV were significantly increased (p less than 0.05 and p less than 0.025 respectively).

Doppler left ventricular inflow time-velocity curves have been extensively used to evaluate left ventricular filling. The reproducibility of the technique, however, has been rarely assessed, its temporal variability being, presently, unknown. In order to define the temporal reproducibility of Doppler parameters of left ventricular filling, 10 normal subjects were studied at 3 different times (baseline, after 24 hours, and after 1 week). No parameter changed significantly during 1 week follow-up. When variability, however, was expressed as percent changes relative to baseline, values ranging from 5.7% to 25% were found, the largest variability being associated with acceleration-deceleration parameters (p less than 0.001). In conclusion temporal variability of parameters obtained from Doppler diastolic mitral time-velocity curves is acceptable in homogeneous groups of subjects. When the technique, however, is used for serial evaluation of ventricular filling in a single patient, the specific temporal variability of the parameter considered must be taken into account before any variation can be ascribed to real hemodynamic changes.

The angiographic morphology of coronary lesions is often completely ignored in the prognostic and decision-making process related to patients with coronary disease. We performed this study to evaluate the possibility of identifying complex or complicated atherosclerotic lesions by means of routine diagnostic coronary arteriography, and to assess their prevalence in the different syndromes of ischaemic heart disease. From an overall group of 200 successive cases studied using coronary angiography, 111 patients with significant coronary artery disease in whom a "culprit lesion" could be identified were retrospectively selected. The angiographic morphology of coronary lesions was defined according to an original classification as: 1) simple stenosis, 2) complex lesion, 3) thrombosis. Of the 111 patients, 36 had been studied for stable angina, 31 for unstable angina, 10 for a non-Q wave myocardial infarction, 34 for transmural infarction. The clinical groups did not show any significant differences when compared on the basis of number of vessels involved and degree of narrowing of the ischaemia-producing artery. Significant differences were found when angiographic morphology was analyzed. In stable angina 78% of ischaemia producing lesions appeared as simple stenoses, while 92% of the unstable or more severely ischaemic patients exhibited complicated lesions (p less than 0.001). In unstable angina and non-Q infarction a complex lesion was present respectively in 71% and 60% of the cases; clear-cut intraluminal thrombosis was demonstrated in 23% of unstable angina, in 30% of non-Q wave infarction and in 39% of transmural infarction (p less than 0.05).(ABSTRACT TRUNCATED AT 250 WORDS)