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Biomedical subjects

M A Gatzoulis

Publications and source records attributed to M A Gatzoulis.

At least 19 recordsLinked to original sources
PubMed2006

The right ventricle in congenital heart disease.

In patients with congenital heart disease the right ventricle (RV) may support the pulmonary (subpulmonary RV) or the systemic circulation (systemic RV). During the last 50 years evidence is accumulating that RV dysfunction develops in many of these patients and leads to considerable morbidity and mortality. Therefore RV function in certain groups of congenital heart disease patients needs close surveillance and timely and appropriate intervention to optimise outcomes. Despite major progress being made, assessing the RV either in the subpulmonary or the systemic circulation remains challenging, often requiring a multi-imaging approach and expertise (echocardiography, magnetic resonance imaging, nuclear and occasionally invasive assessment with angiography). This review discusses the implications of volume and pressure loading of the RV in the context of congenital heart disease and describes the most relevant imaging modalities for monitoring RV function.

Echocardiography↗
PubMed2005

Intrinsic histological abnormalities of aortic root and ascending aorta in tetralogy of Fallot: evidence of causative mechanism for aortic dilatation and aortopathy.

BACKGROUND: Dilatation of the aortic root is a known feature in tetralogy of Fallot (TOF) patients with pulmonary stenosis (PS) or pulmonary atresia (PA). We hypothesized that intrinsic histological abnormalities of the aortic wall present since infancy are an important causative factor leading to aortic root dilatation. METHODS AND RESULTS: We examined the aortic histology of 17 cases with TOF and PS/PA from our cardiac morphology archive and compared them with a control group of normal aortas. Measured circumference of the aortic root at the sinotubular junction and at the ascending aorta was indexed to the left ventricle. Aortic walls were studied by light microscopy with the use of various stains. Seventeen TOF cases (7 with PS, 10 with PA) including 7 infants, 2 children, and 8 adults were compared with 11 hearts with normal aorta. Aortic root circumference to left ventricular index and ascending aortic circumference to left ventricular index were 1.24+/-0.25 and 1.37+/-0.24, respectively, in the TOF group versus 0.89+/-0.10 and 0.88+/-0.11, respectively, in the control group (P<0.001). Histological changes of grade 2 or 3 were present in 29% (medionecrosis), 82% (fibrosis), 35% (cystic medial necrosis), and 59% (elastic fragmentation) in the ascending aorta of the TOF group. Histology grading scores were significantly higher in the TOF group (median score, 7; range, 1 to 12) compared with normal controls (median score, 2; range, 0 to 6) and correlated with the ascending aortic circumference to left ventricular index (r=0.525, P=0.03). CONCLUSIONS: There are marked histological abnormalities in the aortic root and ascending aortic wall of patients with TOF present from infancy, suggesting a causative mechanism for subsequent aortic root dilatation.

Adult↗
PubMed2004

Joint outpatient clinics for the adult with congenital heart disease at the district general hospital: an alternative model of care.

Congenital heart disease (CHD) affects approximately 250,000 adults in the UK. Most of these patients would benefit from specialized follow-up. However, there is at present a significant shortfall of specialized tertiary care expertise and facilities for this growing cardiovascular field in the UK and around the world. We aimed to report our experience with a joint adult CHD clinic run in a district general hospital with regular input from the local cardiology team and a visiting adult CHD specialist. In total, 148 patients aged 33.6+/-14.1 years were seen once or more in 12 clinics over the study period (September 1999 to January 2003). Diagnostic case mix consisted of 2.9% complex, 67.9% moderate and 29.2% minor cases of CHD. Twenty percent of patients visited the counterpart tertiary center for additional investigations (mostly MRI) and 8% for intervention (with no operative mortality). There was one death during the study period giving an overall mortality of 0.2%/year. Patients were referred to the clinic from tertiary centres, the local cardiology and paediatric clinics and with time from obstetric and community sources. Nonattendance rates were relatively low, comparing favourably with tertiary care. This model of joint care for the adult CHD patient at a general district hospital with regular onsite specialized input appears to be effective and highlights the need for additional resource allocation to provide optimal care for these patients. Our data may be useful in future planning for CHD services.

Adult↗
PubMed2004

Massive pulmonary artery thrombosis with haemoptysis in adults with Eisenmenger's syndrome: a clinical dilemma.

Although the frequency of haemoptysis in Eisenmenger's syndrome is well recognised, the high prevalence of pulmonary artery thrombus has been newly appreciated through the growing use of non-invasive imaging. Three patients with Eisenmenger's syndrome with haemoptysis are reported who underwent computed tomography pulmonary angiography and cardiovascular magnetic resonance. Each patient was found to have aneurysmal dilatation of the right pulmonary artery with large laminar thrombus. These cases illustrate a rising clinical problem in this special population-that is, how to treat and prevent large pulmonary artery thrombosis in the setting of haemoptysis. The authors discuss their approach to these cases and the known literature.

Adult↗
PubMed2003

Pacing for adult patients with left atrial isomerism: efficacy and technical considerations.

AIM: Left atrial isomerism (LAI) is an uncommon congenital heart disease, which is combined with a range of intracardiac abnormalities. Atrial arrhythmia is common in LAI. We hypothesized that permanent pacing will decrease arrhythmia recurrence in adult patients with LAI. METHODS AND RESULTS: Patients with LAI and permanent pacing were identified from the Royal Brompton Adult Congenital Heart Database. Hospital records were reviewed, and patients' current status was assessed with clinic reviews including standard and Holter ECGs, and transthoracic echocardiograms. Four patients who underwent permanent pacing at the age of 27.5+/-11.7 years fulfilled inclusion criteria, namely permanent pacing in the setting of LAI. All 4 had sick sinus syndrome-with sustained supraventricular arrhythmia in 3-requiring several hospitalizations for arrhythmia management prior to pacing. All 4 patients remained free of clinical arrhythmia and hospital admission at a mean follow-up of 67.0+/-59.5 months from pacing. The latest ECG revealed paced rhythm in all. NYHA functional class remained stable. There were no pacemaker related complications at implantation or during follow-up. CONCLUSION: Adults with LAI benefit from pacemaker implantation, which reduces recurrence of supraventricular tachycardia and maintains atrioventricular synchrony. Future studies need to determine the preferred pacemaker mode and optimal timing for pacing.

Adolescent↗
PubMed2003

Supraventricular arrhythmia before and after surgical closure of atrial septal defects: spectrum, prognosis and management.

UNLABELLED: Supraventricular arrhythmias are often observed in patients before and after atrial septal defect repair. Although several papers report different incidences of sustained supraventricular arrhythmias, postoperative 'incisional' macroreentrant tachycardias have not been systematically investigated. METHODS: We reviewed 136 consecutive patients (79 female, 57 male, mean age 36.8+/-17.8 years) who underwent atrial septal defect repair at our institutions between January 1990 and January 1999. Coexisting valve disease requiring surgical intervention was noted in 13 patients (9.5%). The mean follow-up period was 78.8+/-30.1 months. RESULTS: Sustained supraventricular arrhythmias occurred in 12 patients (8.8%) before surgery (atrial fibrillation in 11 patients). Using multivariate analysis the occurrence of arrhythmia significantly correlated with the presence of coexisting heart disease (P< 0.001) and age at surgery (P=0.011) After surgery sustained supraventricular arrhythmias were recorded in 16 patients (11.7%). Eleven of them had atrial fibrillation, permanent in 8 cases, 4 'incisional' macroreentrant atrial tachycardia and 1 atrioventricular re-entry tachycardia. There was a significant correlation between pre and postoperative arrhythmia (P< 0.001). Two of the 4 patients with macroreentrant atrial tachycardia underwent successful radiofrequency catheter ablation, whereas the arrhythmia was controlled medically in the remaining 2 patients. CONCLUSIONS: Atrial fibrillation remains the most frequent form of arrhythmia before and after surgical closure of atrial septal defects in adulthood, and relates to age at the time of repair and coexisting heart disease. Incisional macroreentrant atrial tachycardia is an identifiable, albeit less common, form of tachycardia, which can be treated by transcatheter ablation.

Adolescent↗
PubMed2003

Exercise systolic blood pressures are of questionable value in the assessment of the adult with a previous coarctation repair.

BACKGROUND: The role of exercise testing in the follow up of adults with a coarctation repair is unclear. Exercise induced systolic hypertension has been advocated as an indication for further investigation; however, the value of exercise testing in this role has been questioned, especially in paediatric populations. OBJECTIVE: To assess the value of resting and exercise blood pressure measurements in a cohort of adults with repaired coarctation. SETTING: Tertiary referral centre for adult congenital cardiac disease. PATIENTS: 56 patients (33 male, 23 female) with a previous coarctation repair, and 33 age matched controls. MAIN OUTCOME MEASURES: Resting and exercise blood pressures, including arm-leg systolic blood pressure gradients; standard echocardiographic measurements of left ventricular mass, aortic root diameter, and repair site gradient. RESULTS: The coarctation cohort had higher resting upper limb blood pressures than the controls (systolic: 129.7 v 120.7 mm Hg, p = 0.014; diastolic: 76.8 v 72.2 mm Hg, p = 0.02). Mean resting arm-leg systolic blood pressure gradient was also higher, at 3.6 v -2.2 mm Hg, p = 0.027. However, there were no differences between the peak exercise systolic blood pressures of the two groups. Peak exercise systolic blood pressure did not correlate with resting arm-leg blood pressure gradient (r = 0.24, p = 0.13) or with repair site gradient (r = 0.14, p = 0.39). Resting upper limb systolic blood pressure and resting arm-leg systolic blood pressure gradient were related to repair site gradient (r = 0.33, p = 0.03, and r = 0.47, p = 0.002). CONCLUSIONS: Measurements of upper limb blood pressure during exercise are of limited value in the assessment of the post-repair coarctation patient. If routine exercise testing is to be advocated in this population it must be for another indication.

Adult↗
PubMed2001

Impact of pulmonary valve replacement on arrhythmia propensity late after repair of tetralogy of Fallot.

BACKGROUND: Chronic pulmonary regurgitation after repair of tetralogy of Fallot (TOF) may lead to right ventricular dilatation, which may be accompanied by ventricular tachycardia and sudden death. We aimed to examine the effects of pulmonary valve replacement (PVR) on (1) certain electrocardiographic markers predictive of monomorphic ventricular arrhythmia and sudden death and (2) sustained atrial flutter/fibrillation and monomorphic ventricular tachycardia. METHODS AND RESULTS: We studied 70 patients who underwent PVR for pulmonary regurgitation and/or right ventricular outflow tract obstruction late after repair of TOF. Maximum QRS duration and QT dispersion were measured from standard ECGs before PVR and at the latest follow-up. Arrhythmia was defined as sustained atrial flutter/fibrillation or sustained monomorphic ventricular tachycardia. Concomitant intraoperative electrophysiological mapping and/or cryoablation were performed in 9 patients (60%) with preexisting ventricular tachycardia and 6 patients (50%) with preexisting atrial flutter. QRS duration remained unchanged in the study group (P=0.46), but it was significantly prolonged (P<0.001) in a comparable group of patients with repaired TOF who did not undergo PVR. At a mean follow-up of 4.7 years, the incidence of ventricular tachycardia diminished from 22% to 9% (P<0.001), and atrial flutter/fibrillation decreased from 17% to 12% (P=0.32). Intraoperative ablation prevented recurrence of preexisting tachyarrhythmia (0 of 15 patients). CONCLUSIONS: PVR in patients with previous TOF repair and chronic pulmonary regurgitation leads to stabilization of QRS duration and, in conjunction with intraoperative cryoablation, to a decrease in the incidence of preexisting atrial and ventricular tachyarrhythmia. When applicable, this combined approach should be used in patients late after repair of TOF.

Adolescent↗
PubMed2001

The Fontan procedure in adults.

SETTING: Tertiary adult congenital cardiac referral centre. DESIGN: Retrospective cross sectional analysis. OBJECTIVES: To report our 20 year experience with adult Fontan operations, and to compare late outcome in patients with single ventricle with definitive aortopulmonary or cavopulmonary shunt palliation. PATIENTS AND MAIN OUTCOME MEASURES: Patients older than 18 years undergoing Fontan operation between 1 January 1982 and 31 December 1998 were identified. Mortality and late outcome were derived from hospital records. These patients were compared with a cohort of 50 adults with single ventricle who had not undergone a Fontan operation. RESULTS: 61 adults, median age 36 years (range 18-47 years), with a median follow up of 10 years (range 0-21 years) were identified. Actuarial survival was 80% at one year, 76% at five years, 72% at 10 years, and 67% at 15 years. Compared with before the Fontan operation, more patients were in New York Heart Association (NYHA) functional class I or II at the latest follow up (80% v 58%, p < 0.001). Systolic ventricular function deteriorated during follow up such that 34% had moderate to severe ventricular dysfunction at the latest follow up compared with 5% before Fontan (p < 0.001). Arrhythmia increased with time (10% before Fontan v 57% after 10 years, p < 0.001). Fontan patients had improved NYHA functional class, ventricular function, atrioventricular regurgitation, and fewer arrhythmias than the non-Fontan group at the latest follow up. CONCLUSION: The Fontan operation in adults has acceptable early and late mortality. Functional class, systolic ventricular function, atrioventricular regurgitation, and arrhythmia deteriorate late after surgery but to a lesser degree than in non-Fontan patients with a single ventricle.

Adolescent↗
PubMed2000

Right and left ventricular systolic function late after repair of tetralogy of Fallot.

Right ventricular (RV) dysfunction has adverse effects on long-term outcome in patients with repaired tetralogy of Fallot (TOF). We employed serial radionuclide angiography (RNA) to examine RV and left ventricular (LV) systolic function in adults late after TOF repair and its relation to clinical outcome. We reviewed 10-year records of 95 patients (53 men) with TOF followed in our clinic (mean age at repair 12.6 +/- 10.5 years, mean age at last follow-up 37.7 +/- 9.8 years) who underwent at least 2 RNAs between 1987 and 1997. Most patients were well by the end of the study (80% were New York Heart Association class I, 17% were class II, and 3% were in class III). Sixteen patients experienced sustained tachyarrhythmias (8 had atrial; 8 patients had ventricular). One patient died suddenly. Fifteen patients underwent RV outflow reoperations (15 underwent pulmonary valve replacement; 7 had relief of RV outflow obstruction); RV systolic function during exercise in these 15 patients was significantly impaired before and returned to similar levels after surgery, compared with the rest of the patients. Overall, RV and LV function remained stable in the whole group at a mean interval of 5.7 +/- 2.2 years between first and last RNA. This group of closely followed adults with TOF remained well over 10 years with a low incidence of sudden death and stable RV and LV systolic function, despite a relatively large number of RV outflow reoperations. Aggressive intervention for right-sided hemodynamic abnormalities may have contributed to this outcome. Preserved ventricular function may herald a favorable long-term outlook in this group.

Adult↗