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Percutaneous edge-to-edge mitral valve (MV) repair is extensively used in different pathological MV conditions. Randomized controlled trials have evaluated the role of this technique in both primary (organic) and secondary (functional) mitral regurgitation (MR). Furthermore, recent analyses of these studies have shown the relevance of echocardiographic patient selection in the functional setting of MR, differentiating proportionate MR from disproportionate MR according to the degree of the effective regurgitant orifice area (EROA) related to the left ventricular volume. The haemodynamic impact of MR cannot be univocally measured by echocardiography alone and the aim of our study was to determine how invasive LAP monitoring during percutaneous edge-to-edge MV repair can predict long-term procedural success on top of the echocardiographic assessment by introducing the VCX INDEX and identifying haemodynamic variables with direct influence on filling pressures. The VCX INDEX, reflecting the impact of MR, is calculated by dividing the difference between v wave (ventricular systole in the left atrial pressure, LAP, or in the pulmonary capillary wedge pressure, PCWP, waveform) and the mean minimum LAP or mean minimum PCWP (mean between minimum LAP or minimum PCWP, x wave, and a/c wave) by systolic arterial pressure (SAP): (v wave – mean minimum LAP or mean minimum PCWP)/SAP. 85 patients at our centres underwent invasive intracardiac pressure monitoring either measuring LAP during percutaneous edge-to-edge MV repair or PCWP during right heart catheterization. Median VCX INDEX was 0.1 (Q1 0.05, Q3 0.16). The study population was further analysed according to the echocardiographic aetiology of MR: in the organic MR subgroup median VCX INDEX was 0.08 (Q1 0.05, Q3 0.14), in the functional proportionate MR subgroup median VCX INDEX was 0.07 (Q1 0.03, Q3 0.13) and in the functional disproportionate MR subgroup median VCX INDEX was 0.11 (Q1 0.06, Q3 0.19). 20 patients were deemed inoperable by the Heart Team and no further intervention was performed, while 65 patients underwent percutaneous edge-to-edge MV repair with MitraClip device and VCX INDEX was recalculated after the procedure. Median post-MitraClip VCX INDEX was 0.04 (Q1 0.02, Q3 0.07) and a subanalysis based on the echocardiographic MR aetiology was repeated: median post-MitraClip VCX INDEX was 0.02 in the organic MR subgroup (Q1 0.01, Q3 0.05), 0.03 in the functional proportionate MR subgroup (Q1 0.02, Q3 0.07) and 0.05 in the functional disproportionate MR subgroup (Q1 0.03, Q3 0.07). Median VCX INDEX in patients who did not undergo MitraClip implantation was 0.07 (Q1 0.04, Q3 0.12). The variation of VCX INDEX when comparing pre- and post-procedural invasive pressure assessment gives an insight of MitraClip’s favourable haemodynamic effect in terms of VCX INDEX reduction in the treated subgroup of the study and how the intervention has a comparable haemodynamic impact between different echocardiographic MR aetiologies. Further studies are needed to explore the incremental diagnostic role in the decision-making process as well as the prognostic value of the VCX INDEX in patients undergoing percutaneous edge-to-edge MV repair.

Background Hypertrophic cardiomyopathy (HCM) has a complex pathophysiology and heterogeneous phenotypic expression. In obstructive HCM with significant mitral regurgitation (MR), MitraClip device implantation reduces MR severity and symptoms. There are no data regarding MitraClip implantation in patients with non-obstructive HCM and significant MR. Case summary A 78-year-old woman with non-obstructive HCM and significant functional MR (3+) was admitted to our centre for dyspnoea and episodes of pre-syncope under light stress. Transthoracic and transoesophageal echocardiography showed a normal left ventricular ejection fraction and normal right heart pressures, an inverted mitral filling pattern, and a central prevalent jet (A2-P2 origin) of MR. Exercise echocardiography performed to verify exercise tolerance was interrupted at the 50-W stage due to severe hypotension and pre-syncope. After transcatheter edge-to-edge repair using the MitraClip system, the patient exhibited a reduction in MR Grade from 3+ to 1+. Follow-up up to 1-year post-procedure revealed noticeable improvements in exercise tolerance and symptoms. There were no further episodes of pre-syncope. Discussion In non-obstructive HCM, the pathophysiological role of MR in symptom generation is unknown. In this patient, we speculated that significant MR contributed to the mechanisms responsible for severe hypotension and pre-syncope during exercise. A reduction in MR after MitraClip implantation was associated with symptomatic improvements. Our findings further highlight the potential utility of the exercise stress test in therapeutic decision-making for patients with non-obstructive HCM and MR.

We describe the case of a 47-year-old man presenting with inferior acute myocardial infarction complicated by complete atrioventricular block and cardiogenic shock, who underwent primary angioplasty and rheolytic thrombectomy with AngioJet and subsequent evaluation by optical coherence tomography. Optical coherence tomography is a rather novel technique in the management of coronary disease, and as far as we know, this is the first report about optical coherence tomography imaging after AngioJet thrombectomy. Moreover, we provide a brief discussion on advantages and disadvantages of this imaging modality on the basis of current practice.

BACKGROUND The offspring of hypertensive families are characterized by higher arterial blood pressure values and a depressed autonomic control of heart rate. The present study aimed to verify whether these differences are associated with a different genotype distribution of functionally relevant polymorphisms of the alpha- and beta-adrenergic receptor (AR) genes. METHODS We selected 109 age- and sex-matched young normotensive subjects with (FH+, n = 56) and without (FH-, n = 53) a family history of hypertension who underwent evaluation of arterial pressure; 24-h electrocardiogram monitoring to assess time-domain parameters of autonomic heart rate control [i.e. mean RR interval (NN), SD of RR intervals (SDNN) and mean square root of the differences of consecutive RR intervals (rMSSD)]; spectral baroreflex sensitivity measurement; and echo-Doppler to assess diastolic function and left ventricular mass. They were also characterized for the following polymorphisms by means of polymerase chain reaction-restriction fragment polymorphism analysis: Arg492Cys in the alpha1a-AR; Del301-303 in the alpha2b-AR; Ser49Gly and Arg389Gly in the beta1-AR; and the 5' leader cistron Arg19Cys, Arg16Gly and Gln27Glu in the beta2-AR. RESULTS FH+ individuals showed a higher systolic pressure, a lower SDNN and a greater isovolumic relaxation time compared to normotensive offspring. No differences were found between the two groups when genotype distribution of the studied polymorphisms was considered. Subjects carrying alpha1a-AR Cys492 allelic variant showed lower values of NN, SDNN and rMSSD, independent of age, gender and body mass index. CONCLUSIONS The functionally relevant polymorphisms of alpha2b-, beta1- and beta2-AR genes are not associated with a family history of essential hypertension. The Arg492Cys polymorphism of the alpha1a-AR gene, although not associated with a family history of hypertension, was strongly related to autonomic control of heart rate.

Abnormalities in the natriuretic peptide system could play a key role in the genesis of hypertension. We evaluated the associations between a family history of hypertension, cardiovascular phenotype and allelic variants of Npr1 and Npr3, two candidate genes that codify for natriuretic peptide receptors.We genotyped 45 young normotensive subjects (19 males, 26.8 +/- 3.7 years) with accurately assessed family history of hypertension (FH+) and 52 (26 males, 26.1 +/- 3.1 years) without (FH-) for the known variants of Npr1 and Npr3 genes, and for a novel length difference (3C/4C) polymorphism at position 15129 in the 3'-untranslated region of the Npr1 gene. Blood pressure, echocardiography and plasma brain natriuretic peptide were assessed.Both the novel Npr1 3C allele (59 versus 33%, P0.001) and the 3C/3C genotype (31 versus 8%; P0.001) were significantly more frequent in FH+ than in FH-. The inverse distribution of the 4C/4C genotype suggested that a casual association was very unlikely. Moreover, the 3C/3C homozygous had significantly higher systolic blood pressure (121.1 +/- 6.3 versus 115.6 +/- 7.8 mmHg in 4C/4C; P0.05) and a longer left ventricular isovolumic relaxation time (67 +/- 10 versus 61 +/- 9 ms; P0.05). The Npr3 C(-55) allele variant was also more frequent in FH+ (88 versus 76%, P0.05), but was not associated with the cardiovascular phenotype.The novel Npr1 gene 3C variant and the Npr3 gene C(-55) allele are associated with hypertensive family history. Moreover, the functional Npr1 3C variant, when homozygous, is also associated with higher systolic blood pressure and prolonged ventricular relaxation.

Autosomal dominant polycystic kidney disease (ADPKD) may present with a variety of extrarenal manifestations. Only 1 case of spontaneous coronary artery dissection has been reported so far in ADPKD. Here we report a case of coronary artery dissection occurring in a 38-year-old woman with ADPKD and an unremarkable medical history. She was admitted to the intensive care unit with acute chest pain. Anterior myocardial infarction was diagnosed by ECG; the patient was treated with thrombolysis with tenecteplase and aspirin, heparin and carvedilol. Then she was referred to the cardiac catheterization laboratory. Coronary angiography revealed a long linear dissection in the middle left descending coronary artery (DCA) with TIMI 1 distal flow. Complete vessel recanalization was achieved by positioning 2 drug-eluting and overlapped stents in the middle DCA and a further proximal stent due to residual proximal dissection, resulting in a TIMI 3 distal flow. There were no procedural complications, and the patient was discharged on day 8. In conclusion, spontaneous coronary artery dissection is a rare cause of myocardial ischemia and infarction. Myocardial infarction in patients without any risk factors for coronary atherosclerosis or in young patients should prompt a diligent search for a possible spontaneous coronary artery dissection. It is suggested that ADPKD may be a predisposing factor for spontaneous coronary artery dissection.

Natriuretic peptide system plays a well-defined role in the regulation of blood pressure and fluid volume. Although the effects of natriuretic peptides (atrial natriuretic peptide, brain natriuretic peptide and C-type natriuretic peptide) are mediated by specific biologic receptors, their plasma level is influenced by clearance receptors. It has been demonstrated that in hypertensive subjects plasma levels of natriuretic peptides are impaired; furthermore peptide receptor polymorphisms have been shown to be significantly associated with hypertension and cardiac hypertrophy. Studying normotensive subjects at high genetic risk of developing hypertension on the basis of family history makes it possible to investigate the role of natriuretic peptide system in the genesis of hypertension. It has been shown that plasma atrial and ventricular natriuretic peptide levels are significantly reduced in normotensive subjects with a family history of hypertension. Our study is the first one showing association among positive family history of essential hypertension and natriuretic peptide receptor polymorphisms. We identified a novel insertion/deletion polymorphism at position 15,129 in the 3'-untranslated region (3'-UTR) of NPRA receptor mRNA. The NPRA gene deletion variant is associated with hypertensive family history and higher systolic blood pressure. The "deletion 15129" variant might participate in the functional impairment of natriuretic peptide system defining an increased genetic susceptibility to hypertension.