Left atrial septal pouch in a six-year-old child: echocardiographic and magnetic resonance view.

In a 6-year-old child patient, transthoracic echocardiography revealed a large saccular structure (with anechogenic content) in the left atrium, near the fossa ovalis, and diagnosis of the left atrial septal pouch was made. The left atrial septal pouch is a kangaroo pouch-like structure on the left side of the interatrial septum, opened into the left atrial cavity without a connection between the left and right atria. It occurs when the foramen ovale is absent but the septum primum and septum secundum are only partially fused. The left atrial septal pouch is believed to be present in 47% of population. In many cases, the pocket on the atrial septum is small and it could not be detected by transthoracic echocardiography. Our description is uncommon because we diagnosed a very large septal pouch. Based on our knowledge, this is the youngest reported case of the left atrial septal pouch and the longest follow-up described in this type of congenital heart malformation.

Current challenges in endovascular therapy for thoracic aneurysms.

Surgical treatment of thoracic aortic aneurysms can be associated with high mortality and complication rates, resulting in prolonged hospital stay. Endovascular techniques for treating thoracic aortic disease have been rapidly embraced, showing limited mortality and major adverse events. Its wide availability and relative ease of application has changed and extended management options for thoracic aortic disease, including inpatients deemed unsuitable for open surgery. Rapid technical evolution has allowed it to be applied to even hostile anatomy and complex cases. However, as it is still a young technology, many unknowns remain, including long-term outcomes with respect to conservative treatment and open surgical repair.

Troponin T elevation in acute aortic syndromes: Frequency and impact on diagnostic delay and misdiagnosis.

AIMS: Despite troponin assay being a part of the diagnostic work up in many conditions with acute chest pain, little is known about its frequency and clinical implications in acute aortic syndromes (AASs). In our study we assessed frequency, impact on diagnostic delay, inappropriate treatments, and prognosis of troponin elevation in AAS. METHODS AND RESULTS: Data were collected from a prospective metropolitan AAS registry (398 patients diagnosed between 2000 and 2013). Cardiac troponin test, using either standard or high sensitivity assay, was performed according to standard protocol used in chest pain units. Troponin T values were available in 248 patients (60%) of the registry population; the overall frequency of troponin positivity was 28% (ranging from 16% to 54%, using standard or high sensitivity assay respectively, p = 0.001). Troponin positivity was frequently associated with acute coronary syndromes (ACS)-like electrocardiogram findings, and with a twofold increased risk of long in-hospital diagnostic time (odds ratio (OR) 1.92, 95% confidence interval (CI) 1.05-3.52, p = 0.03). The combination of positive troponin and ACS-like electrocardiogram abnormalities resulted in a significantly increased risk of in-hospital delay/coronary angiography/antithrombotic therapy due to a misdiagnosis of ACS (OR 2.48, 95% CI 1.12-5.54, p = 0.02). However, troponin positivity was not associated with in-hospital mortality (OR 1.63, 95% CI 0.86-3.10, p = 0.131). CONCLUSIONS: Troponin positivity was a frequent finding in AAS patients, particularly when a high sensitivity assay was employed. Abnormal troponin values were strongly associated with ACS-like electrocardiogram findings and with in-hospital diagnostic delay but apparently they did not influence in-hospital mortality.

Predictors of nonsimultaneous interventricular delay at cardiac resynchronization therapy optimization.

AIM: Cardiac resynchronization is a well tolerated and effective therapy for heart failure, but 30% of patients still do not respond to biventricular pacing. Optimization of device settings, in particular interventricular delay value, represents a plausible target for improving these results, but available literature is discordant. We aimed our study at the identification of the best suitable candidates to interventricular delay optimization. METHODS: A total of 77 consecutive patients with optimized drugs therapy underwent clinical, echocardiographic and electrocardiographic evaluation before and after 6 months from implantation of a biventricular defibrillator in accordance to current guidelines. In each patient, atrioventricular and interventricular delay values were optimized at predischarge with echocardiogram. RESULTS: The only predictor of an optimized interventricular delay value different from simultaneous (i.e. standard shipment setting), at both univariate and multivariate analyses, was a QRS duration greater than 160 ms (odds ratio 22.958; P = 0.003) with a sensitivity of 70.9%. CONCLUSION: Candidates to cardiac resynchronization therapy with a basal QRS greater than 160 ms have a higher chance of requiring echo-guided tailoring of interventricular delay value. A strategy based on these data can potentially improve device programming, reducing by one-third the need for optimization, according to our findings, and at the same time avoid unnecessary time-consuming procedures.

Effects of myocardial fibrosis assessed by MRI on dynamic left ventricular outflow tract obstruction in patients with hypertrophic cardiomyopathy: a retrospective database analysis.

BACKGROUND: While implications of myocardial fibrosis on left ventricular (LV) function at rest have been studied in hypertrophic cardiomyopathy (HCM), the pathophysiological consequences on dynamic LV outflow tract (LVOT) gradient have so far not been investigated in detail. OBJECTIVE: To evaluate the influence of myocardial fibrosis, detected by MRI as late-gadolinium enhancement (LGE), on LVOT gradient in HCM. DESIGN: Retrospective database analysis. SETTING: A single Italian cardiomyopathies referral centre. PATIENTS: Seventy-six HCM patients with normal ejection fraction at rest. INTERVENTIONS: Patients underwent cardiac MR and performed bicycle exercise echocardiogram within a month. RESULTS: LGE was present in 54 patients (71%), ranging from 0.2% to 32.4% of LV mass. There was a weak correlation between the amount of fibrosis and LVOT gradient variation during exercise in the overall population (r=-0.243, p=0.034) and a stronger correlation in patients with obstructive HCM at rest (r=-0.524, p=0.021). Patients with an LVOT gradient increase ≥50 mm Hg during exercise had a significantly lesser extent of fibrosis than those with an increase <50 mm Hg (0.7% (IQR 0-2.4) vs 3.2% (IQR 0.2-7.4), p=0.006). The extent of fibrosis was significantly lower among the highest quartiles of LVOT gradient increase (p=0.009). CONCLUSIONS: In patients with HCM and normal ejection fraction at rest, myocardial fibrosis was associated with a lower increase in LVOT gradient during exercise, probably due to a lesser degree of myocardial contractility recruitment. This negative association was more evident in patients with an obstructive form at rest.

Effects of cardiac resynchronisation therapy on dilated cardiomyopathy with isolated ventricular non-compaction.

OBJECTIVE: To compare the effects of cardiac resynchronisation therapy (CRT) on left ventricular (LV) reverse remodelling in patients with dilated cardiomyopathy (DCM) associated or not with isolated ventricular non-compaction (IVNC). METHODS AND RESULTS: 52 patients with heart failure, candidates for CRT, were recruited: 20 patients with IVNC associated with DCM (IVNC-DCM) without other coexisting cardiac diseases and 32 patients with DCM without IVNC matched for age, gender, body surface area and LV systolic function. Standard and contrast echocardiography were used to assess LV volumes and function and to optimise visualisation of the endocardial border at baseline and at 6 months' follow-up. Patients with heart failure were subsequently classified as CRT negative responders, non-responders, responders or super-responders based on different LV reverse remodelling 6 months after CRT implantation. Different types of CRT response were observed in IVNC-DCM and DCM patients. In particular, in IVNC-DCM patients the percentage of super-responders was significantly higher than for patients with DCM (60% vs 28%, respectively, p = 0.023). In addition, the number of IVNC segments had a trend towards reduction with respect to baseline (4 (3-6)) at 6 months' follow-up (3 (1-5); p = 0.067). Finally, in IVNC-DCM, the patients with a higher number of IVNC segments at baseline (>4) were more likely to be responders or super-responders than patients with ≤ 4 IVNC segments (p = 0.003). CONCLUSIONS: Patients with IVNC-DCM had greater LV reverse remodelling after CRT than patients with DCM. The greater the area of non-compaction (higher number of IVNC segments) the greater the chance of achieving CRT response and greater LV reverse remodelling.

Echocardiography in aortic diseases: EAE recommendations for clinical practice.

Echocardiography plays an important role in the diagnosis and follow-up of aortic diseases. Evaluation of the aorta is a routine part of the standard echocardiographic examination. Transthoracic echocardiography (TTE) permits adequate assessment of several aortic segments, particularly the aortic root and proximal ascending aorta. Transoesophageal echocardiography (TOE) overcomes the limitations of TTE in thoracic aorta assessment. TTE and TOE should be used in a complementary manner. Echocardiography is useful for assessing aortic size, biophysical properties, and atherosclerotic involvement of the thoracic aorta. Although TOE is the technique of choice in the diagnosis of aortic dissection, TTE may be used as the initial modality in the emergency setting. Intimal flap in proximal ascending aorta, pericardial effusion/tamponade, and left ventricular function can be easily visualized by TTE. However, a negative TTE does not rule out aortic dissection and other imaging techniques must be considered. TOE should define entry tear location, mechanisms and severity of aortic regurgitation, and true lumen compression. In addition, echocardiography is essential in selecting and monitoring surgical and endovascular treatment and in detecting possible complications. Although other imaging techniques such as computed tomography and magnetic resonance have a greater field of view and may yield complementary information, echocardiography is portable, rapid, accurate, and cost-effective in the diagnosis and follow-up of most aortic diseases.

Interventricular delay optimization: a comparison among three different echocardiographic methods.

AIM OF THE STUDY: To compare three different methods for obtaining interventricular-(VV) interval optimization. METHODS: A total of 30 patients undergoing cardiac resynchronization therapy (CRT) were enrolled. All the patients underwent VV-interval optimization performed with three different echocardiographic methods at seven different settings: simultaneous right (RV) and left (LV) ventricular pacing, LV stimulation only, LV preexcitation (LV+20,+40,+60 ms, respectively), RV preexcitation (RV+20 and+40 ms, respectively). Optimal VV delay was selected by: (1) measuring the aortic velocity time integral (VTI method); (2) measuring the time to maximum delay between septal and lateral longitudinal motion in the four-chamber view (velocity method); and (3) measuring the segment with maximal temporal difference of peak circumferential strain in short-axis view at papillary muscles level, (strain method). Velocity and strain methods measurements were obtained relying on two-dimensional ultrasound border tracking algorithm thus providing angle-independent measurements. RESULTS: Immediately after CRT, VTI, maximum peak circumferential strain delay and maximum septal-to-lateral delay were significantly improved (P < 0.001). Particularly, VV-interval optimization determined a further improvement of these indices as compared to the other VV settings (P < 0.001). Furthermore, a substantial concordance was found between the optimal VV interval obtained according to the VTI method and velocity method (k = 0.68), between the optimal VV interval obtained according to the VTI method and strain method (k = 0.63); and between the optimal VV interval obtained according to the velocity method and strain method (k = 0.71). CONCLUSIONS: VV-interval optimization was shown to determine a further benefit beyond CRT. A significant concordance was present between VV programming based on different echocardiographic methods.

Prognostic implications of the Doppler restrictive filling pattern in hypertrophic cardiomyopathy.

The Doppler echocardiographic pattern of restrictive left ventricular (LV) filling has proved to be an important predictor of clinical course and prognosis in dilated cardiomyopathy. However, the relation between restrictive filling pattern and clinical course has not been systematically investigated in hypertrophic cardiomyopathy (HC). We assessed the prognostic implications of the Doppler restrictive filling pattern in 239 consecutive patients with HC in whom Doppler measurements of LV filling had been systematically recorded at initial evaluation and during follow-up. Restrictive LV filling was identified in 14 patients (5.9%) at initial evaluation and developed in 22 (9.2%) during follow-up. A close relation was identified between restrictive filling pattern and end-stage HC, with patients with restrictive filling showing a sixfold increase in risk of developing end-stage HC (hazard ratio 6.25, 95% confidence interval 1.90 to 20.57, p = 0.003). Over a median follow-up of 9.7 years, 22 patients (9.2%) died suddenly or received appropriate cardioverter-defibrillator interventions, and 54 (22.6%) had HC-related death or underwent heart transplantation. In a set of univariate and multivariate analyses including each of the generally accepted risk factors for cardiac death in HC, the restrictive filling pattern was a strong and independent marker of increased risk (hazard ratio for sudden cardiac events 3.51, 95% confidence interval 1.37 to 8.95, p = 0.009; hazard ratio for HC-related death or heart transplantation 3.54, 95% confidence interval 1.91 to 6.57, p <0.001) compared to patients without restrictive filling. In conclusion, in our study cohort, the Doppler pattern of restrictive LV filling proved to be a strong predictor of sudden death and HC-related death, independently of other markers for unfavorable prognosis in this disease.

Stress Doppler echocardiography in relatives of patients with idiopathic and familial pulmonary arterial hypertension: results of a multicenter European analysis of pulmonary artery pressure response to exercise and hypoxia.

BACKGROUND: This large, prospective, multicentric study was performed to analyze the distribution of tricuspid regurgitation velocity (TRV) values during exercise and hypoxia in relatives of patients with idiopathic and familial pulmonary arterial hypertension (PAH) and in healthy control subjects. We tested the hypothesis that relatives of idiopathic/familial PAH patients display an enhanced frequency of hypertensive TRV response to stress and that this response is associated with mutations in the bone morphogenetic protein receptor II (BMPR2) gene. METHODS AND RESULTS: TRV was estimated by Doppler echocardiography during supine bicycle exercise in normoxia and during 120 minutes of normobaric hypoxia (FIO(2)=12%; approximately 4500 m) in 291 relatives of 109 PAH patients and in 191 age-matched control subjects. Mean maximal TRVs were significantly higher in PAH relatives during both exercise and hypoxia. During exercise, 10% of control subjects but 31.6% of relatives (P<0.0001) exceeded the 90% quantile of mean maximal TRV seen in control subjects. Hypoxia revealed hypertensive TRV in 26% of relatives (P=0.0029). Among control subjects, TRV at rest was not related to age, sex, body mass index, systemic blood pressure, smoking status, or heart rate. Within kindreds identified as harboring deleterious mutations of the BMPR2 gene, a hypertensive TRV response occurred significantly more often compared with those without detected mutations. CONCLUSIONS: Pulmonary hypertensive response to exercise and hypoxia in idiopathic/familial PAH relatives appears as a genetic trait with familial clustering, being correlated to but not caused by a BMPR2 mutation. The suitability of this trait to predict manifest PAH development should be addressed in long-term follow-up studies.

Left ventricular function after ST-elevation myocardial infarction in patients treated with primary percutaneous coronary intervention and abciximab or tirofiban (from the Facilitated Angioplasty with Tirofiban or Abciximab [FATA] Trial).

Abciximab therapy during primary percutaneous coronary intervention (PCI) has shown to ameliorate left ventricular (LV) function recovery in patients with ST elevated myocardial infarction. High-dose bolus tirofiban has similar effect on platelet inhibition. Whether this is associated with comparable efficacy on LV function recovery remains unclear. We sought to evaluate the impact on LV function of high-dose bolus tirofiban or abciximab in patients undergoing primary PCI with the predictors of favorable (> or = 50%) LV ejection fraction (EF) and LV function recovery at 30 days. We studied 314 patients (abciximab n = 154; tirofiban n = 160) undergoing primary PCI in the randomized Facilitated Angioplasty with Tirofiban or Abciximab (FATA) Trial. LVEF was assessed within 48 hours and at 30 days after primary PCI. In patients with systolic dysfunction at baseline, LV function recovery was defined by either increase of LVEF > or = 10% compared with baseline or LVEF > or = 50%. Similar LVEF was observed in the 2 groups postprocedure (abciximab 49.7 +/- 10.1% vs tirofiban 49.3 +/- 10.1%, p = 0.9) and at 30 days (abciximab 53.1 +/- 9.8% vs tirofiban 52.5 +/- 10.2%, p = 0.6). Independent predictors of 30-day LVEF > or = 50% were preprocedure Thrombolysis In Myocardial Infarction flow class >0 (odds ratio = 2.4, 95% confidence interval 1.32 to 4.34), anterior location (odds ratio = 0.25, 95% confidence interval 0.15 to 0.42), and age (odds ratio = 0.97, 95% confidence interval 0.95 to 0.99). Preprocedure Thrombolysis In Myocardial Infarction flow grade >0 was the only predictor of LV function recovery (odds ratio = 6.73, 95% confidence interval 2.69 to 16.88). In conclusion, this study showed no difference in LV function recovery in patients undergoing primary PCI treated either with abciximab or high-dose bolus tirofiban. Preprocedure Thrombolysis In Myocardial Infarction flow grade >0 seems to be the most important predictor of favorable LVEF and LV function recovery at 30 days.

Exercise stress echocardiography is superior to rest echocardiography in predicting left ventricular reverse remodelling and functional improvement after cardiac resynchronization therapy.

AIMS: Cardiac resynchronization therapy (CRT) improves functional capacity and survival in heart failure. However, one-third of patients fail to respond to CRT. Resting left ventricular (LV) dyssynchrony assessed by echocardiography (ECHO) showed discordant results in identifying CRT responders. LV dyssynchrony can totally change during exercise. Aim of this study was to evaluate whether exercise dyssynchrony could select responders to CRT. METHODS AND RESULTS: Sixty-four patients scheduled for CRT implantation performed bicycle exercise ECHO in semi-supine position on an exercise tilting table before and 6 months after CRT implantation. Tissue Doppler imaging (TDI) was acquired both at rest and during exercise to detect LV mechanical dyssynchrony. Predictive values for CRT response were 70% for rest TDI and 89% for exercise TDI (P = 0.01). Exercise LV dyssynchrony was the only parameter independently associated with follow-up improvement of rest ejection fraction and LV volume during multivariable analysis (P < 0.001). Functional improvement at 6-min walking test was statistically higher in patients with exercise dyssynchrony (P = 0.005), and not different considering rest dyssynchrony (P = 0.30). CONCLUSION: Exercise intraventricular dyssynchrony assessed by exercise TDI ECHO is a strong independent predictor of CRT response. It could be used to select candidates for CRT, thus reducing ineffective implantations of biventricular pacemakers.

Heart transplantation in hypertrophic cardiomyopathy.

Heart transplantation (HT) is the sole therapeutic option for selected patients with hypertrophic cardiomyopathy (HC) and refractory heart failure. However, the results of HT have not been systematically investigated in HC. We assessed the pathophysiologic profile of HT candidates and the outcome after transplantation in 307 patients with HC consecutively evaluated at our tertiary referral center from 1987 to 2005; follow-up was 9.9+8.2 years. Outcome of recipients with HC was compared with that of 141 patients who underwent transplantation for idiopathic dilated cardiomyopathy at our center over the same period. Of 21 patients with HC who entered the transplantation list, 20 had end-stage evolution with systolic dysfunction and 1 had an extremely small left ventricular cavity with impaired filling and recurrent cardiogenic shock during paroxysmal atrial fibrillation. Of 33 study patients with HC who showed end-stage evolution during follow-up, the 23 who were included on the waiting list or died from refractory heart failure (2 patients) were significantly younger than the 10 patients who remained clinically stable (37+/-14 vs 57+/-17 years, p=0.004). Of the 21 HT candidates, 18 underwent transplantation during follow-up. In heart transplant recipients, 7-year survival rate was 94% and not different from that of the 141 patients who received transplants for idiopathic dilated cardiomyopathy (p=0.66). In conclusion, long-term outcome after HT in patients with HC is favorable and similar to that of patients with idiopathic dilated cardiomyopathy. In patients with end-stage HC, young age is associated with more rapid progression to refractory heart failure.

Left ventricular versus biventricular pacing: a randomized comparative study evaluating mid-term electromechanical and clinical effects.

BACKGROUND: Although left ventricular (LV) pacing has been proposed as an alternative to biventricular (BIV) pacing for heart failure (HF) patients, few comparative data are available on the electromechanical effects of these pacing modalities at mid-term follow-up. AIM: To investigate the clinical and echocardiographic effects of LV versus BIV pacing in a mid-term randomized study. METHODS: After implantation of a device with LV/BIV pacing capabilities, 22 patients with chronic HF and left bundle branch block were randomized to LV or BIV pacing. Patients were assessed both preimplantation and after 3 months by clinical examination, ECG and echocardiography with pulsed tissue Doppler imaging. RESULTS: At 3 months LV pacing improved clinical parameters, LV ejection fraction (+5%, range 5-8%, P = 0.007) and intraventricular dyssynchrony (-40 ms, range -50 to -15 ms, in septal to lateral delay, P = 0.008) to a similar extent to BIV pacing. A decrease in interventricular mechanical delay (-25 ms, range -40 to -5 ms, P = 0.008) and QRS duration (-28 ms, range -40 to -5 ms, P = 0.008) was observed in BIV, but not in LV patients. CONCLUSION: In this pilot evaluation, LV pacing appeared to be associated with clinical benefits similar to BIV pacing at mid-term follow-up, and this was combined with an improvement in intraventricular dyssynchrony, regardless of variations in interventricular dyssynchrony and QRS duration. Echocardiographic evaluation of intraventricular dyssynchrony seems to be an appropriate method for assessing the chronic response to LV pacing.

Cardiac resynchronization therapy: variations in echo-guided optimized atrioventricular and interventricular delays during follow-up.

BACKGROUND: Relatively few data are available on long-term echocardiographic optimization of atrioventricular (AV) and interventricular (VV) delay programming in cardiac resynchronization therapy (CRT). We assessed variations in optimized AV and VV delays during long-term follow-up. METHODS: Thirty-seven consecutive heart failure patients received Doppler echocardiographic optimization of AV and VV delay within 48 hours from CRT device implantation, at 6 months and at 12 months (the last for the first enrolled 14 patients). RESULTS: After implantation, median optimized AV delay was 100 ms (range, 45 ms); VV optimization led to simultaneous biventricular activation in 4 patients, left ventricular preactivation in 17 patients and right ventricular preactivation in 16 patients. At 12 months median AV delay decreased to 85 ms (23 ms) (P < 0.05 vs. baseline). With respect to previous assessment, VV delay variations > or =40 ms were observed in 41% of the patients at 6 months and in 57% of the tested patients at 12 months. A nonconcordance (by Kappa test) of optimized VV delays was found between each new assessment and the previous one. VV delay optimization was associated with significant (P < 0.001) increases in aortic velocity time integral both at baseline and during follow-up. CONCLUSIONS: Echocardiographic optimization of AV and VV delay is associated with broad intraindividual variability during follow-up. A new assessment of optimized VV delays during long-term follow-up reveals a nonconcordance with previous values and provides increases in forward stroke volume.

Frequency, determinants, and clinical relevance of acute coronary syndrome-like electrocardiographic findings in patients with acute aortic syndrome.

We investigated frequency/characteristics of acute coronary syndrome-like (ACS-like) electrocardiographic (ECG) profiles among patients with a final diagnosis of acute aortic syndrome (AAS), and explored pathophysiologic determinants and prognostic relevance within each Stanford subtype. We blindly reviewed presentation electrocardiograms of 233 consecutive patients with final diagnosis of AAS (164 Stanford type A) at a regional treatment center. Prevalence of ACS-like ECG findings was 27% (type A, 26%, type B, 29%); most were non-ST-elevation myocardial infarction-like. Patients with ACS-like ECG findings more often had coronary ostia involvement (p=0.002), pleural effusion (p=0.02), significant aortic regurgitation (p=0.01), and troponin positivity (p=0.001). ACS-like ECG profile in type A disease was independently associated with coronary ostia involvement (odds ratio [OR] 5.27, 95% confidence interval [CI] 1.75 to 15.88). ACS-like ECG profile predicted in-hospital mortality (OR 2.90, 95% CI 1.24 to 6.12), as did age (each incremental 10-year: OR 1.59, 95% CI 1.14 to 2.22), and syncope at presentation (OR 2.90, 95% CI 1.16 to 7.24). In conclusion, about 25% of our AAS patients (in either Stanford subtype) presented ACS-like ECG patterns-often with non-ST-elevation myocardial infarction characteristics-which could cause misdiagnosis. ACS-like ECG profile was associated with more complicated disease, and in type A disease was a strong independent predictor of in-hospital mortality.

Impact of transverse aortic arch hypoplasia after surgical repair of aortic coarctation: an exercise echo and magnetic resonance imaging study.

BACKGROUND: We sought to assess the impact of persistent hypoplasia of the transverse aortic arch (TAA) after repair of aortic coarctation (AoC), on blood pressure response to exercise, left ventricular (LV) hypertrophy and presence of collateral circulation. METHODS: 34 consecutive patients with end-to-end repair of AoC (age at repair 3.2+/-2.5 years) underwent exercise echocardiography and magnetic resonance imaging (MRI) at 24+/-7 years of age (range 11.3 to 44.6 years). Systolic Doppler pressure gradient (SPG) across the descending aorta and blood pressure at the right arm were measured at baseline and every minute throughout all exercise. Magnetic resonance imaging was used to measure LV mass index, presence and amount of collateral flow, and the diameters of the aortic isthmus and TAA indexed to the diameter of the diaphragmatic. RESULTS: Aortic isthmus index was higher than that of the TAA (p=0.006). We observed LV hypertrophy in 15 patients (45%) and presence of collateral circulation in 14 (41%). Eighteen patients (53%) had an abnormal blood pressure response to exercise. Patients with abnormal pressure response to exercise had smaller TAA index (p=0.0005), but similar aortic isthmus index (p=0.09). They also had higher exercise SPG (p<0.0001), higher LV mass index (p<0.0001) and prevalence of LV hypertrophy (p=0.007), higher prevalence of collateral circulation (p<0.0001) and a higher amount of collateral flow (p<0.0001). TAA index, but not aortic isthmus index, correlated with exercise blood pressure (r=-0.59, p=0.003), exercise SPG (r=-0.70, p=0.0005), amount of collateral flow (r=-0.74, p=0.0002) and LV mass index (r=-0.68, p=0.0007). CONCLUSIONS: After repair of AoC, hypoplasia of the TAA may be responsible for abnormal blood pressure response to exercise, persistence of collateral circulation and LV hypertrophy.

Different types of cardiomyopathy associated with isolated ventricular noncompaction.

Although mainly described in the context of dilated and hypokinetic left ventricles, it is unclear whether isolated ventricular noncompaction (IVNC) is a distinct cardiomyopathy, a subtype of dilated cardiomyopathy, or a morphogenetic disorder. To investigate the spectrum of cardiomyopathies associated with IVNC, children and adults with stringent echocardiographic diagnoses of IVNC were reviewed. Seventy-three patients (12 children aged <15 years) seen since 1994 satisfied stringent echocardiographic criteria for IVNC. Sixty-five patients (89%; 11 children) had dilated cardiomyopathy, 2 adults had clear-cut hypertrophic cardiomyopathy, 1 adult had restrictive cardiomyopathy (to the investigators' knowledge, the first reported example of this particular association), and 5 patients (1 child) had normal left ventricular morphology and function. In conclusion, knowledge that IVNC can co-exist with restrictive and hypertrophic cardiomyopathy (in addition to the dilated form) supports the concept that IVNC is a morphologic trait rather than a distinct cardiomyopathy. This knowledge should be taken into account during echocardiographic examination and encourage the use of contrast echocardiography (and magnetic resonance) and could also orient molecular biology studies.