Exploring the Continuum of Hypertrophic Cardiomyopathy-From DNA to Clinical Expression.

The concepts underlying hypertrophic cardiomyopathy (HCM) pathogenesis have evolved greatly over the last 60 years since the pioneering work of the British pathologist Donald Teare, presenting the autopsy findings of "asymmetric hypertrophy of the heart in young adults". Advances in human genome analysis and cardiac imaging techniques have enriched our understanding of the complex architecture of the malady and shaped the way we perceive the illness continuum. Presently, HCM is acknowledged as "a disease of the sarcomere", where the relationship between genotype and phenotype is not straightforward but subject to various genetic and nongenetic influences. The focus of this review is to discuss key aspects related to molecular mechanisms and imaging aspects that have prompted genotype-phenotype correlations, which will hopefully empower patient-tailored health interventions.

Successful surgical repair of a triple cardiac rupture using modified cohesive double patch technique.

Introduction The association between both left and right ventricular free wall ruptures (FWR) and post-infarction anterior ventricular septal defect (VSD) is an exceptional situation. Case report We present the case of a patient who developed a VSD and two FWRs (of the left and right ventricle, respectively) shortly after the onset of an anterior AMI. We surgically closed this complex rupture using the cohesive double patch technique with two Teflon patches combined with an infarct exclusion technique. The left and right ventricular patches were attached cohesively to the septal wall and the infarcted area was excluded without reducing the left ventricular cavity. Conclusion Association between post-infarction ventricular septal rupture and both left and right free wall ruptures are a very rare and dangerous situation. The modified cohesive double patch technique associated the modified Cooley technique seems to be the correct surgical solution.

An unusual association: right atrial myxoma and severe left ventricular dysfunction. Case report and review of the literature.

Myxomas comprise 50% of all benign cardiac tumors in adults, with the right atrium as their second most frequent site of origin. Surgical resection is the only effective therapeutic option for patients with these tumors. The association between right atrial myxomas and severe left ventricular systolic dysfunction is extremely rare and makes treatment even more challenging. This was the case for our patient, a 47-year-old male with a right atrial mass and a severely impaired left ventricular function, with a 20% ejection fraction. Global enlargement of the heart was also noted, with moderate right ventricular dysfunction. The tumor was successfully excised using the on-pump beating heart technique, with an immediate postoperative improvement of the left ventricular ejection fraction to 35%. The technique proved useful, with no increased risk to the patient.

Impact of secondary mitral valve chordal cutting on valve geometry in obstructive hypertrophic cardiomyopathy with marked septal hypertrophy.

AIMS: In patients with obstructive hypertrophic cardiomyopathy (HCM) and mild septal thickness undergoing myectomy, resecting fibrotic anterior mitral leaflet (AML) secondary chordae moves the mitral valve (MV) away from the outflow tract and ejection flow, reducing the need for a deep septal excision. Aim of the present study was to assess whether chordal resection has similarly favourable effects in patients with important hypertrophy, who represent the majority of patients with obstructive HCM. METHODS AND RESULTS: The MV position in the ventricular cavity, assessed from echocardiography as AML-annulus ratio, was compared before and after chordal resection in 150 consecutive HCM patients with important (≥20 mm) and 62 with mild (≤19 mm) septal thickness undergoing myectomy. Preoperatively, MV position was displaced towards the septum to a similar extent in both groups. Postoperatively, AML-annulus ratio increased of an equal degree in both groups, from 0.43 ± 0.05 to 0.55 ± 0.06 (P < 0.001) a 28% increase, and from 0.43 ± 0.06 to 0.55 ± 0.06 (P < 0.001) a 26% increase, respectively, indicating a similar MV shift away from the outflow tract. When AML-annulus ratio was compared in the study cohort and 124 normal subjects, MV position was within normal range in <4% of patients preoperatively and normalized in >50% postoperatively. CONCLUSIONS: In obstructive HCM, displacement of the MV apparatus into the outflow tract interferes with the ejection flow. Resection of fibrotic secondary chordae moves the MV apparatus away from the outflow tract and enlarges the outflow area independently of septal thickness, facilitating septal myectomy by reducing the need for a deep muscular excision.

Budget impact analysis of a bovine pericardial aortic bioprosthesis versus mechanical aortic valve replacement in adult patients with aortic stenosis in Romania.

AIMS: An analysis of the budget impact of using a bovine pericardial aortic bioprosthesis (BPAB) or a mechanical valve (MV) in aortic stenosis (AS) patients in Romania. MATERIALS AND METHODS: A decision-tree with a partitioned survival model was used to predict the financial outcomes of using either a BPAB (the Carpentier-Edwards Perimount Magna Ease Valve) or MV in aortic valve replacement (AVR) procedure over a 5-year period. The budget impact of various resource consumption including disabling strokes, reoperations, minor thromboembolic events, major bleeding, endocarditis, anticoagulation treatment and monitoring, and echocardiogram assessments were compared for both types of valves. One-way sensitivity analyses (OWSA) were conducted on the input costs and probabilities. RESULTS: The use of BPAB compared to MV approaches budget neutrality due to incremental savings year-on-year. The initial surgical procedure and reoperation costs for BPAB are offset by savings in acenocoumarol use, disabling strokes, major bleeding, minor thromboembolic events, and anticoagulation complications. The cost of the initial procedure per patient is 460 euros higher for a BPAB due to the higher valve acquisition cost, although this is partially offset by a shorter hospital stay. The OWSA shows that the total procedure costs, including the hospital stay, are the primary cost drivers in the model. LIMITATIONS: Results are limited by cost data aggregation in the DRG system, exclusion of costs for consumables and capital equipment use, possible underestimation of outpatient complication costs, age-related variations of event rates, and valve durability. CONCLUSIONS: Adopting BPAB as a treatment option for AS patients in Romania can lead to cost savings and long-term economic benefits. By mitigating procedure costs and increasing anticoagulation treatment costs, BPAB offers a budget-neutral option that can help healthcare providers, policymakers, and patients alike manage the growing burden of AS in Romania.

Transaortic Shallow Septal Myectomy and Cutting of Secondary Fibrotic Mitral Valve Chordae-A 5-Year Single-Center Experience in the Treatment of Hypertrophic Obstructive Cardiomyopathy.

BACKGROUND: Anomalies of the mitral apparatus have been shown to contribute to left ventricular outflow obstruction in patients with hypertrophic cardiomyopathy (HCM). We report our 5-year single-center experience with a shallow myectomy procedure associated with transaortic mitral valve repair in a cohort of HCM patients. METHODS: We studied 83 consecutive patients who underwent surgical treatment of symptomatic left ventricular outflow obstruction. In all study patients, a transaortic shallow septal myectomy was performed. Fibrous or muscular structures connecting the papillary muscles to the septum or free wall were resected, and fibrotic secondary chordae of the anterior mitral valve were cut selectively. RESULTS: We report one death (1.2%) during hospitalization, no iatrogenic ventricular septal defects, and two (2.4%) mitral valve replacements. At discharge, no patients were in New York Heart Association (NYHA) Class III/IV, from 49 (59%) preoperatively. Mean maximal septal thickness decreased from 24 ± 6 to 16 ± 3 mm. Mean outflow gradient decreased from 93 ± 33 to 13 ± 11 mmHg. Grade 3 or 4 mitral regurgitation was noticed in one patient postoperatively, from 32 (39%) before surgery. CONCLUSIONS: Shallow septal myectomy associated with secondary mitral valve chordal cutting and papillary muscle mobilization provided excellent results offering adequate treatment of outflow obstruction.

Unique case of primary malignant fibrous histiocytoma of the right ventricle with moderator band involvement.

Malignant primary tumors of the heart are very rare and in most cases are located in the left side of the heart, but involvement of the moderator band of the right heart has not yet been described in the literature. We report the case of a 22-year-old woman who presented after an episode of transient sudden loss of consciousness. The transthoracic echocardiography (TTE) and transesophageal echocardiography evaluations showed an echogenic mass that originated in the right ventricle and protruded into the tricuspid valve during systole. While operating on the patient, we found a sessile tumor that originated exclusively from the moderator band of the right ventricle. The tumor was completely resected through the tricuspid valve. An immunohistochemistry examination of the tumor confirmed the diagnosis of a malignant fibrous histiocytoma (MFH) (undifferentiated pleomorphic sarcoma). To our knowledge, this case is the first of moderator band involvement among the 50 cases of primary MFH of the heart that have been reported in the literature. After a year and a half, TTE, computed tomography (CT), and positron emission tomography CT (PET-CT) showed the absence of cardiac tumor, a normal tricuspid function, and no metastasis. The completeness of the surgical resection and the absence of local recurrence >1 year after surgery are encouraging signs for the patient's outcome.

Gender Disparities in the Prevalence, Awareness, Treatment, and Control of High Blood Pressure.

The gender effects in arterial hypertension (HT) epidemiology remain poorly clarified to date. We present an up-to-date review of the data regarding gender disparities in HT's prevalence, awareness, treatment, and control. Based on the data from three consecutive national-representative SEPHAR (Study for the Evaluation of Prevalence of Hypertension and Cardiovascular Risk in Romania) surveys conducted between 2005 and 2016, we provide insights into gender differences in HT's epidemiology and their 11- years the evolutionary trend in a high-CV risk European country. Our data displays gender effects in different age-dependent epidemiological patterns in terms of hypertension prevalence, awareness, treatment, and control, mainly due to hormonal status. Hypertension's prevalence is higher in younger men and older women. Although women are more often aware of their hypertensive condition and receive more often antihypertensive treatment, BP control is lower in older women compared to men of the same age, mainly due to a higher treatment side-effect rate. There is no solid evidence that different antihypertensive drugs exhibit different effects in lowering BP values between genders. In high CV risk European countries like Romania, if all the influencing conditions remain similar to those in the past 11 years, gender discrepancies in terms of HT's prevalence will diminish over time, awareness and treatment of hypertension will continue to be higher in females than in men, with an upward trend of BP control predicted only for women, while in men HT treatment control rate is expected to stagnate.

High-normal blood pressure and related cardiovascular risk factors prevalence in the Romanian adult population: insights from the SEPHAR III study.

High normal blood pressure (HNBP) is associated with an increased risk of incident high blood pressure (HBP) and of cardiovascular diseases (CVD). To estimate the prevalence of HNBP and related cardiovascular risk factors, a representative sample of 1970 Romanian adults was enrolled in SEPHAR III survey (Study for the Evaluation of Prevalence of Hypertension and Cardiovascular Risk in Romania). All were evaluated for blood pressure values and by a 71-item questionnaire, anthropometric measurements, together with extensive evaluation for target organ damage, blood, and urine sample collection. Prevalence of HNBP was 11% [45.1% had HBP, 43.9% normal BP (NBP)]. HNBP individuals were older (51.14 ± 17.13 years) than subjects with NBP (40.5 ± 15.96 years) but younger than those with HBP (55.79 ± 15.68 years), p < 0.0001 (95% CI 18-85, respectively 18-91). Values of weight, waist circumference, body mass index, total and LDL cholesterol, triglycerides, fasting blood glucose, glycosylated hemoglobin (HbA1c), uric acid, serum creatinine, glomerular filtration rate estimate by CKD-EPI equation, urinary albumin to creatinine ratio, intimae-media thickness, arterial stiffness measurements and diastolic dysfunction, indexed left ventricular mass, interventricular septum and posterior left ventricle wall thickness, left atrial volume, and LA dilatation were significantly higher in HNBP subjects than in NBP. Our study showed that individuals with HNBP represent ~11% and most of them had an elevated total cardiovascular risk. It is essential to educate the public and health care providers to be aware of these individuals and of steps that should be taken to treat modifiable cardiovascular risk factors.

The brachio-brachial arteriovenous fistula: mid-term results.

OBJECTIVE: To evaluate the mid-term results of the brachio-brachial arteriovenous fistula in patients without adequate superficial venous circulation in the upper limb. METHODS: Retrospective analysis included 49 patients, in whom a brachio-brachial fistula had been created in an end-to-side configuration. After the maturation period (1 month), the brachial vein was transposed into the subcutaneous tissue. Follow-up study was performed in patients with functional brachio-brachial fistula after the superficialization. RESULTS: Forty-nine patients underwent 49 brachio-brachial fistula constructions. All fistulas were functional. One month after surgery, 40 (81.6%) of these patients had a functional fistula, but in only 39 (79.6%) cases was the fistula suitable for hemodialysis (HD) following transposition to subcutaneous tissue. During the 1-month maturation period, the fistula became occluded in nine patients, and in one case the vein was permeable, so the fistula was functional, but too small to permit HD. Seventeen patients developed temporary edema of the forearm during the first month, in three cases the edema was extended to the entire arm, but no other complications were associated with the procedure. Follow-up lasted 18.0 +/- 11.1 (3-37) months, during which 7/39 patients presented with fistula occlusion. Three patients died and another three were out of the study for various reasons. CONCLUSIONS: The brachio-brachial fistula is a good alternative to prosthetic grafts in patients without superficial venous circulation in the upper limb.

Congenital Muscular Mitral-Aortic Discontinuity Identified in Patients With Obstructive Hypertrophic Cardiomyopathy.

BACKGROUND: The mitral valve is often structurally abnormal in hypertrophic cardiomyopathy (HCM). However, the mechanisms responsible for these abnormalities remain controversial. In 2016 we identified, at myectomy, muscular mitral-aortic discontinuity in 5 young patients with obstructive HCM. OBJECTIVES: This study sought to confirm our preliminary findings and assess the prevalence of muscular mitral-aortic discontinuity in obstructive HCM. METHODS: At our center, from January 2017 to April 2018, the area between the anterior mitral leaflet and aortic valve was inspected at myectomy in 106 consecutive patients with HCM. RESULTS: Muscular mitral-aortic discontinuity was identified in 28 (26%) patients and was significantly more common in younger than older patients (age 39 ± 13 years vs. 58 ± 11 years; p < 0.001). Muscular discontinuity was present in each of 6 patients aged <30 years but only 1 (2.7%) of 37 aged ≥60 years. Pathogenic sarcomere mutations were identified in 22 (79%) of 28 patients with and 24 (31%) of 78 without discontinuity (p < 0.001) and were associated with discontinuity independently of age (p = 0.021). Discontinuity mean length was 7.3 mm and was inversely related to age (p = 0.022). At echocardiography, the anterior mitral leaflet was longer in patients with than those without discontinuity (34 ± 4 mm vs. 29 ± 5 mm; p < 0.001). CONCLUSIONS: We report, for the first time, muscular mitral-aortic discontinuity in HCM. At myectomy, a long muscular discontinuity displaced the anterior mitral leaflet toward the apex in most young patients, was significantly associated with sarcomere mutations independent of age, and was extremely uncommon in older patients. These findings suggest that a long muscular mitral-aortic discontinuity could predispose to the development of outflow obstruction in young patients with sarcomere mutations.

Yield of Rare Variants Detected by Targeted Next-Generation Sequencing in a Cohort of Romanian Index Patients with Hypertrophic Cardiomyopathy.

BACKGROUND: The aim of this study was to explore the rare variants in a cohort of Romanian index cases with hypertrophic cardiomyopathy (HCM). METHODS: Forty-five unrelated probands with HCM were screened by targeted next generation sequencing (NGS) of 47 core and emerging genes connected with HCM. RESULTS: We identified 95 variants with allele frequency < 0.1% in population databases. MYBPC3 and TTN had the largest number of rare variants (17 variants each). A definite genetic etiology was found in 6 probands (13.3%), while inconclusive results due to either known or novel variants were established in 31 cases (68.9%). All disease-causing variants were detected in sarcomeric genes (MYBPC3 and MYH7 with two cases each, and one case in TNNI3 and TPM1 respectively). Multiple variants were detected in 27 subjects (60%), but no proband carried more than one causal variant. Of note, almost half of the rare variants were novel. CONCLUSIONS: Herein we reported for the first time the rare variants identified in core and putative genes associated with HCM in a cohort of Romanian unrelated adult patients. The clinical significance of most detected variants is yet to be established, additional studies based on segregation analysis being required for definite classification.

Current management and surgical advances in patients with hypertrophic obstructive cardiomyopathy.

Hypertrophic cardiomyopathy (HCM) is a genetic disease and the most frequent primary cardiomyopathy, affecting 1:500 of the general population. Integrated multimodality imaging, including transthoracic echocardiography, 2- and 3­dimensional transesophageal echocardiography, stress echocardiography, and cardiac magnetic resonance, has provided answers to questions on the management of HCM, leading to standardized protocols. The late 1990s brought the news of a nonsurgical treatment of obstruction in HCM. It is now increasingly evident that septal ablation cannot address all the mechanisms of the left ventricular outflow tract (LVOT) gradient, especially mitral valve involvement. According to American and European guidelines, surgical septal myectomy is the current gold standard treatment. However, deep septal myectomy requires specific operator and institutional experience; therefore, it should not be performed in small community hospitals but only in centers of excellence for HCM treatment. The so-called Ferrazzi technique involves cutting the fibrotic secondary chordae of the mitral valve (MV) and thus helps avoid a deep myectomy by moving the anterior mitral leaflet and the coaptation point of the MV posteriorly away from the septum. This technique, together with careful mobilization of the papillary muscles, helped us achieve excellent results since November 2015, with no mortality, resolution of the LVOT gradient, and MV preservation in all 72 patients. Owing to recent advances in the surgical treatment of hypertrophic obstructive cardiomyopathy, addressing not only the septum but also the MV, the procedure of a deep myectomy has been simplified and mitral regurgitation adequately corrected.

Role of Preoperative Cardiovascular Magnetic Resonance in Planning Ventricular Septal Myectomy in Patients With Obstructive Hypertrophic Cardiomyopathy.

In obstructive hypertrophic cardiomyopathy (HC), extreme heterogeneity of septal morphology makes septal myectomy particularly challenging. Although cardiovascular magnetic resonance (CMR) reconstructs ventricular anatomy with high spatial resolution, CMR is not used systematically to plan preoperatively septal myectomy. In this study, we report our results with using CMR to plan the extent of septal excision in 112 consecutive HC patients who subsequently underwent myectomy. Depth and length of the myectomy planned at CMR were compared with those of the septal muscle excised in a single piece in all patients. Anterior septum maximal thickness at CMR was 22 ± 5 mm and excised muscle thickness 9 ± 3 mm. Planned myectomy length was 35 ± 11 mm (range 17 to 65) and excised muscle length 38 ± 10 mm (range 10 to 70), indicating extension of septal resection to mid-cavity. Thickness and length of the planned myectomy showed a significant correlation with the excised muscle (R2 = 0.345; p <0.001; and R2 = 0.358; p <0.001, respectively). Deep septal crypts were identified at CMR in 12(11%) patients, preventing muscle excision from areas at increased risk of iatrogenic septal defect. Large aberrant muscle bundles that could decrease mid-cavity dimension were identified at CMR and excised in 26(23%) patients. In the 55 patients with postoperative CMR, qualitative comparison of pre and postoperative ventricular morphology showed a smooth and apically extended myectomy. In conclusion, CMR planning of septal myectomy provided high resolution images of septal morphology and allowed us to perform a standardized and apically extended septal excision that was associated with favorable outcome. Our novel approach could make myectomy more accessible to cardiovascular surgeons.

Combined cardiac-neurosurgical treatment of acute aortic dissection, stroke, and coma.

Coma or stroke with secondary brain malperfusion is usually considered a strong contraindication for emergent surgical treatment of acute aortic dissection. Herein, we present the case of a 30-year-old woman who presented with sudden left hemiplegia and level-7 coma on the Glasgow Coma Scale. Transthoracic echocardiography showed type A aortic dissection. Although the patient was unable to communicate, her family approved an emergency Bentall operation. She regained consciousness but developed anisocoria and Glasgow Coma Scale level-4 coma 30 hours after the operation. Computed tomography showed massive cerebral infarction with hernia of the uncus gyri hippocampi. Emergency surgical cerebral decompression was performed. The patient survived; after 1 year, she had full mental acuity and minor left motor sequelae.

Intraoperative Diagnosis of Anderson-Fabry Disease in Patients With Obstructive Hypertrophic Cardiomyopathy Undergoing Surgical Myectomy.

Importance: Diagnostic screening for Anderson-Fabry cardiomyopathy (AFC) is performed in the presence of specific clinical red flags in patients with hypertrophic cardiomyopathy (HCM) older than 25 years. However, left ventricular outflow tract obstruction (LVOTO) has been traditionally considered an exclusion criteria for AFC. Objective: To examine a series of patients diagnosed with HCM and severe basal LVOTO undergoing myectomy in whom the diagnosis of AFC was suspected by the cardiac surgeon intraoperatively and confirmed by histological and genetic examinations. Design, Setting, and Participants: This retrospective analysis of patients undergoing surgical septal reduction strategies was conducted in 3 European tertiary referral centers for HCM from July 2013 to December 2016. Patients with a clinical diagnosis of obstructive HCM referred for surgical management of LVOTO were observed for at least 18 months after the procedure (mean [SD] follow-up, 33 [14] months). Main Outcomes and Measures: Etiology of patients with HCM who underwent surgical myectomy. Results: From 2013, 235 consecutive patients with a clinical diagnosis of HCM underwent septal myectomy. The cardiac surgeon suspected a storage disease in 3 patients (1.3%) while inspecting their heart samples extracted from myectomy. The mean (SD) age at diagnosis for these 3 patients was 42 (4) years; all were male. None of the 3 patients presented with extracardiac features suggestive of AFC. All patients showed asymmetrical left ventricular hypertrophy, with maximal left ventricular thickness in the basal septum (19-31 mm), severe basal LVOTO (70-120 mm Hg), and left atrial dilatation (44-57 mm). Only 1 patient presented with late gadolinium enhancement on cardiovascular magnetic resonance at the right ventricle insertion site. The mean (SD) age at surgical procedure was 63 (5) years. On tactile sensation, the surgeon felt a spongy consistency of the surgical samples, different from the usual stony-elastic consistency typical of classic HCM, and this prompted histological examinations. Histology showed evidence of intracellular storage, and genetic analysis confirmed a GLA A gene mutation (p.Asn215Ser) in all 3 patients. Conclusions and Relevance: Screening for AFC should be performed even in the absence of red flags in patients with HCM older than 25 years.

The mechanisms, diagnosis and management of mitral regurgitation in mitral valve prolapse and hypertrophic cardiomyopathy.

Valvular disease is a frequent cardiac pathology leading to heart failure and, ultimately, death. Mitral regurgitation, defined as the inability of the two mitral leaflets to coapt, is a common valvular disease and a self sustained pathology. A better understanding of the mitral valve histological layers provides a better understanding of the leaflet and chordae changes in mitral valve prolapse. Mitral valve prolapse may occur in myxomatous degenerative abnormalities, connective tissue disorders or in sporadic isolated cases. It is the most common mitral abnormality of non-ischemic cause leading to severe surgery-requiring mitral regurgitation. In addition to standard echocardiographic investigations, newly implemented three-dimensional techniques are being used and they permit a better visualisation, from the so-called 'surgical view', and an improved evaluation of the mitral valve. Hypertrophic cardiomyopathy is the most frequent inherited myocardial disease caused by mutations in various genes encoding proteins of the cardiac sarcomere, leading to a marked left ventricular hypertrophy unexplained by other comorbidities. The pathological echocardiographic hallmarks of hypertrophic cardiomyopathy are left ventricular hypertrophy, left ventricular outflow tract obstruction and systolic anterior motion of the mitral valve. The systolic anterior motion of the mitral valve contributes to the development of mitral regurgitation and further narrows the left ventricular outflow tract, leading to more severe symptomatology. Cardiac magnetic resonance imaging accurately measures the left ventricular mass, the degree of diastolic function and it may also be used to distinguish phenotypic variants. The clinical outcome of patients with these pathologies is mostly determined by the selected option of treatment. The purpose of surgical correction regarding mitral valve involvement is to restore valvular competence. Surgery has proven to be the only useful treatment in preventing heart failure, improving symptomatology and reducing mortality. Our approach wishes to enhance the understanding of the mitral valve's involvement in hypertrophic cardiomyopathy and mitral valve prolapse from genetic, haemodynamic and clinical perspectives, as well as to present novelties in the grand field of treatment.

Surgical treatment of a pulmonary artery aneurysm due to a regurgitant quadricuspid pulmonary valve.

We report the case of a patient with a pulmonary artery (PA) aneurysm secondary to a regurgitant quadricuspid pulmonary valve, in which both lesions were successfully repaired. The patient, a 16-year old boy, was known to have had pulmonary regurgitation and progressive dilation of the PA for years. He was operated on when he developed symptoms of effort, a dilated right ventricle and a PA of 55 mm. The quadricuspid pulmonary valve was an intraoperative finding. It had a dilated annulus, two normal cusps, a third with a 'sparrow-nest' configuration and the fourth was severely hypoplastic (which explained the valve regurgitation). The valve was repaired through tricuspidisation of the quadricuspid pulmonary valve (annular plication at the level of the hypoplastic cusp, freeing of excedentary tissue from the 'sparrow-nest' cusp and its reattachment to the plicated annulus). The aneurysm was treated through a reduction pulmonary arterioplasty. Early assessment showed the minimal regurgitation of the valve and a normal diameter PA. The outcome was uneventful, with a stable correction after 44 months of follow-up. To our knowledge, this is the first repair of a quadricuspid pulmonary valve, through tricuspidisation.