Origin and Site of the Tricuspid Regurgitant Jet Determined by Color Flow Imaging in Ebstein's Anomaly

Fifty patients with tricuspid regurgitation due to a variety of causes including rheumatic heart disease, pulmonary hypertension, atrial septal defect, and tricuspid valve endocarditis (group 1); and seven patients with Ebstein's anomaly of the tricuspid valve (group 2) had color Doppler flow imaging performed. The position of the tricuspid annulus and the site of origin of the turbulent tricuspid regurgitant jet were determined from the apical four-chamber view. The site of origin of the tricuspid regurgitant jet was at or within 0.5 cm of the level of the tricuspid annulus in all group 1 patients. In contrast, all patients in group 2 had tricuspid regurgitant jets that originated deep in the right ventricle and at a considerable distance from the tricuspid annulus (3.8 ± 1.4 cm, mean ± SD). The distance from the origin of the tricuspid regurgitant jet to the tricuspid annulus was measured and expressed as a ratio to that of the distance from the right ventricular apex to the tricuspid annulus in groups 1 and 2. This ratio was 0.94 ± 0.2 in group 1 and 0.61 ± 0.10 in group 2 patients. In conclusion, the tricuspid regurgitant jet originates at or near the level of the tricuspid annulus except in patients with Ebstein's anomaly. Origin of the tricuspid regurgitant jet distal to tricuspid annulus is a clue to the diagnosis of Ebstein's anomaly and is particularly helpful in those patients in whom the displaced tricuspid leaflet cannot be convincingly demonstrated on the two dimensional echocardiogram.     

Transesophageal Echocardiography Identification and Validation of Individual Tricuspid Valve Leaflets

The present study demonstrates the usefulness of transesophageal echocardiography in the identification of individual leaflets of the tricuspid valve. Validation was obtained by selecting patients with specific lesions involving individual tricuspid leaflets and correlating the transesophageal echocardiographic findings with surgery.     

Prenatal Diagnosis and Successful Outcome in Neonate of Aorto‐Left Ventricle Tunnel

Aorto‐left ventricular tunnel (ALVT) is an abnormal congenital communication between the ascending aorta and the left ventricle. Prenatal diagnosis is rare and can be misinterpreted as aortic insufficiency on fetal echocardiogram. We present a case of ALVT diagnosed in a fetus who underwent successful early neonatal surgical repair.     

β-Thalassemia Mutations in Western India: Outcome of Prenatal Diagnosis in a Hemoglobinopathies Project

Prenatal diagnosis (PND) is one of the most cost effective preventive methods, but it is available only in the large cities of India. Therefore, we initiated a program that offers PND and allows us to determine the prevalence of various mutations. Pregnant females (n?=?111,426) were screened for hemoglobinopathies using complete blood count (CBC) and high performance liquid chromatography (HPLC). If the female had a hemoglobinopathy, her husband was then tested. If hemoglobinopathies were seen in both partners, a genetic mutation study was performed on the couple. Fetal samples were obtained by either chorionic villus sampling (CVS) in 70.6% or amniocentesis in 29.4%. The study included 282 couples. IVS-I-5 (G?>?C) was the most common mutation in all castes except in the Sindhis and Lohanas, where the 619?bp deletion was the most common. Prenatal testing was informative in 97.9% of the couples. A significant number of couples (41.0%) underwent PND during their first pregnancy. Seven patients with β-thalassemia (β-thal) trait had normal Hb A₂ levels. The Hb A₂ and Hb F values varied significantly (p??T or G?>?A), were present in 81.0% of the couples tested. β-Thalassemia mutation frequency varied among the different castes, underlining the need for evolving a testing strategy that considers the caste system. Targeting antenatal clinics could also prove to be a most cost effective way of preventing hemoglobinopathies.     

Diagnosis and prognostic value of restrictive ventilatory disorders in the elderly: a systematic review of the literature

Although less extensively studied compared to pulmonary obstructive diseases, restrictive lung disease (RLD) is highly prevalent and frequently disabling in the adult and, more, the elderly population. The underlying conditions may be either primarily pulmonary diseases, such as idiopathic pulmonary fibrosis, or non respiratory conditions secondarily affecting the lung, e. g. congestive heart failure, or else conditions affecting the lung expansion, e. g. obesity or rib cage deformity. The diagnosis is frequently based on the measurement of surrogate indexes such as the forced vital capacity (FVC) used as a proxy for total lung capacity (TLC). As a consequence, diagnosis of RLD is often characterized by poor specificity. In the elderly, worsening in the quality of life and poor prognosis are variably, but significantly, associated to RLD, being the underlying condition an important source of variability. Several causes of RLD are preventable and treatable conditions. A prompt identification of these conditions may allow to slow the decline of respiratory reserve and, thus, to preserve both personal independence and resistance to acute respiratory infections. This review gives an update on the latest evidence available on the prevalence and the prognosis of RLD in the elderly. Studies were identified through systematic searches of the electronic database MEDLINE. Reference list of eligible papers were also manually searched.     

Spontaneous Coronary Artery Dissection: Pitfalls of Angiographic Diagnosis and an Approach to Ambiguous Cases

Spontaneous coronary artery dissection (SCAD) is a pathophysiologically distinct cause of acute coronary syndromes (ACS). It is increasingly recognized that optimal management is different from that for atherosclerotic ACS and that a SCAD diagnosis has specific long-term prognostic and therapeutic implications. Accurate diagnosis is therefore essential to ensure the best treatment of patients. At present this relies on the recognition of typical features of SCAD identified on invasive coronary angiography. Although most SCAD can be readily distinguished angiographically from alternative causes of ACS, false positive and false negative diagnoses remain common. In particular, sometimes non-SCAD presentations, including atherothrombosis, takotsubo cardiomyopathy, coronary embolism, coronary vasospasm, contrast streaming, and myocardial infarction with nonobstructive coronary arteries, can mimic angiographic features usually associated with SCAD. The authors present the combined experience from European and US SCAD referral centers reviewing the classical angiographic appearances of SCAD, presenting potential diagnostic pitfalls and exemplars of SCAD mimickers. The authors further review the benefits and limitations of intracoronary imaging in the context of SCAD. Finally, the authors discuss the investigation of ambiguous cases and an approach to minimize misdiagnosis in difficult cases.     

Tricuspid Regurgitation Is Associated With Increased Risk of Mortality in Patients With Low-Flow Low-Gradient Aortic Stenosis and Reduced Ejection Fraction: Results of the Multicenter TOPAS Study (True or Pseudo-Severe Aortic Stenosis)

ObjectivesThis study sought to examine the impact of tricuspid regurgitation (TR) on mortality in patients with low-flow, low-gradient (LF-LG) aortic stenosis (AS) and reduced left ventricular ejection fraction (LVEF).BackgroundTR is often observed in patients with LF-LG AS and low LVEF, but its impact on prognosis remains unknown.MethodsA total of 211 patients (73 ± 10 years of age; 77% men) with LF-LG AS (mean gradient <40 mm Hg and indexed aortic valve area [AVA] ≤0.6 cm²/m²) and reduced LVEF (≤40%) were prospectively enrolled in the TOPAS (True or Pseudo-Severe Aortic Stenosis) study and 125 (59%) of them underwent aortic valve replacement (AVR) within 3 months following inclusion. The severity of AS was assessed by the projected AVA (AVA_proj) at normal flow rate (250 ml/s), as previously described and validated. The severity of TR was graded according to current guidelines.ResultsAmong the 211 patients included in the study, 22 (10%) had no TR, 113 (54%) had mild (grade 1), 50 (24%) mild-to-moderate (grade 2), and 26 (12%) moderate-to-severe (grade 3) or severe (grade 4) TR. During a mean follow-up of 2.4 ± 2.2 years, 104 patients (49%) died. Univariable analysis showed that TR ≥2 was associated with increased risk of all-cause mortality (hazard ratio [HR]: 1.82, 95% confidence interval [CI]: 1.22 to 2.71; p = 0.004) and cardiovascular mortality (HR: 1.85, 95% CI: 1.20 to 2.83; p = 0.005). After adjustment for age, sex, coronary artery disease, AVA_proj, LVEF, stroke volume index, right ventricular dysfunction, mitral regurgitation, and type of treatment (AVR vs. conservative), the presence of TR ≥2 was an independent predictor of all-cause mortality (HR: 1.88, 95% CI: 1.08 to 3.23; p = 0.02) and cardiovascular mortality (HR: 1.92, 95% CI: 1.05 to 3.51; p = 0.03). Furthermore, in patients undergoing AVR, TR ≥3 was an independent predictor of 30-day mortality compared with TR = 0/1 (odds ratio [OR]: 7.24, 95% CI: 1.56 to 38.2; p = 0.01) and TR = 2 (OR: 4.70, 95% CI: 1.00 to 25.90; p = 0.05).ConclusionsIn patients with LF-LG AS and reduced LVEF, TR is independently associated with increased risk of cumulative all-cause mortality and cardiovascular mortality regardless of the type of treatment. In patients undergoing AVR, moderate/severe TR is associated with increased 30-day mortality. Further studies are needed to determine whether TR is a risk marker or a risk factor of mortality and whether concomitant surgical correction of TR at the time of AVR might improve outcomes for this high-risk population.