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Importance: The treatment of neonates with irreparable heart valve dysfunction remains an unsolved problem because there are no heart valve implants that grow. Therefore, neonates with heart valve implants are committed to recurrent implant exchanges until an adult-sized valve can fit., Objective: To deliver the first heart valve implant that grows., Design, Setting, and Participants: Case report from a pediatric referral center, with follow-up for more than 1 year. Participants were a recipient neonate with persistent truncus arteriosus and irreparable truncal valve dysfunction and a donor neonate with hypoxic-ischemic brain injury., Intervention: First-in-human transplant of the part of the heart containing the aortic and pulmonary valves., Main Outcomes and Measures: Transplanted valve growth and hemodynamic function., Results: Echocardiography demonstrated adaptive growth and excellent hemodynamic function of the partial heart transplant valves., Conclusions and Relevance: In this child, partial heart transplant delivered growing heart valve implants with a good outcome at age 1 year. Partial heart transplants may improve the treatment of neonates with irreparable heart valve dysfunction.

There is continued interest in surgical repair of both the congenitally malformed aortic valve, and the valve with acquired dysfunction. Aortic valvar repair based on a geometric approach has demonstrated improved durability and outcomes. Such an approach requires a thorough comprehension of the complex 3-dimensional anatomy of both the normal and congenitally malformed aortic root. In this review, we provide an understanding of this anatomy based on the features that can accurately be revealed by contrast-enhanced computed tomographic imaging. We highlight the complimentary role that such imaging, with multiplanar reformatting and 3-dimensional reconstructions, can play in selection of patients, and subsequent presurgical planning for valvar repair. The technique compliments other established techniques for perioperative imaging, with echocardiography maintaining its central role in assessment, and enhances direct surgical evaluation. This additive morphological and functional information holds the potential for improving selection of patients, surgical planning, subsequent surgical repair, and hopefully the subsequent outcomes.

Background: Pregnancies in women with regurgitant valve lesions are generally considered low risk, but this has not been well studied., Objectives: This study determined the frequency of adverse cardiac events (CEs) in pregnant women with moderate or severe regurgitant valve lesions., Methods: Maternal and fetal outcomes in women with moderate or severe chronic valve regurgitation enrolled in a prospective multicenter study on pregnancy outcomes were examined. Adverse CEs included heart failure, sustained arrhythmias, cardiac arrest, or death. A multivariate logistic regression model was used to identify determinants of CEs in women at the highest risk., Results: Outcomes of 430 pregnancies in women with moderate or severe regurgitant lesions were examined: 145 with mitral regurgitation (MR), 101 with pulmonary regurgitation (PR), 71 with multivalve disease, 73 with tricuspid regurgitation (TR), and 40 with aortic regurgitation (AR). Most women had associated congenital or acquired heart disease. Adverse CEs occurred in 13% of pregnancies: 27% of pregnancies with multivalve disease; 15% with MR; 15% with TR; 5% with AR; and 3% with PR. Maternal mortality was rare. In women with MR, TR, or multivalve disease (n = 289), left ventricular systolic dysfunction (p = 0.001), pulmonary hypertension (p = 0.005), and cardiac events before pregnancy (p < 0.001) were important determinants of CEs during pregnancy., Conclusions: Women with AR and PR are at low risk for cardiac complications during pregnancy. While many women with MR, TR, and multivalve regurgitation do well during pregnancy, additional clinical variables help stratify those at highest risk. This new information will enhance the quality and precision of preconception counseling and pregnancy planning., Competing Interests: Funding Support and Author Disclosures This study was supported by a grant from the Allan E. Tiffin Trust (Toronto General, and Western Hospital Foundation). Dr. Silversides was supported by the Miles Nadal Chair in Pregnancy and Heart Disease (Mount Sinai Hospital Foundation). The authors have reported that they have no relationships relevant to the contents of this paper to disclose., (Copyright © 2021 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Tricuspid valve dysplasia (TVD) is a congenital malformation of the right atrioventricular valve characterized by restricted leaflet motion, annular dilation, and tricuspid regurgitation (TR). Severe cases typically exhibit progressive right-sided congestive heart failure, affecting the quality of life and survival. This article describes a technique for surgical repair of TVD and a case report with long-term follow-up. A 1.5-year-old intact male Labrador retriever with severe TR underwent surgical repair for TVD. Valve repair was performed under cardiopulmonary bypass and consisted of neochord mobilization of the valve leaflets and partial band annuloplasty. Transthoracic echocardiogram performed 5 days after surgery showed mild TR, a 93% decrease in anatomic regurgitant orifice area, and decreased right chamber dimensions. Forty-eight months after repair, the patient was free of clinical signs, did not have a heart murmur, and was receiving no cardiac medications. Based on this case, surgical repair of TVD is feasible with long-term durability, and the outcome suggests that the described technique may be a viable treatment option for patients with severe TVD., (Copyright © 2020 Elsevier B.V. All rights reserved.)

Objective: To summarize the experience of perioperative management strategy of fetal pulmonary valvuloplasty (FPV) for hypoplastic right heart syndrome (HRHS). Methods: In the retrospective study of perioperative data, 13 fetuses of HRHS were treated with FPV in Qingdao Women and Children's Hospital from July 2018 to June 2019. Results: (1) The evaluation indexes of the right ventricle in 13 fetuses before FPV: the mean ratio of tricuspid/mitral annulus, right/left ventricular length, pulmonary/aortic annulus, and tricuspid inflow time/cardiac cycle were 0.81±0.04, 0.56±0.14, 0.69±0.06, and 0.35±0.03, respectively. (2) All pregnant mothers underwent general anesthesia. The basic fetal heart rate was (156±12) beats per minutes before FPV, and 9 fetuses showed bradycardia during the operation, and recovered to normal after drug resuscitation. On the first day after FPV, two cases had bradycardia and pregnancy was terminated. The fluctuation of systolic blood pressure of pregnant mother was less than 20%, and there was no significant difference between preoperative and intraoperative pulse pressure [(36.0±5.6) vs (35.8±6.9) mmHg (1 mmHg=0.133 kPa); t =8.102, P =0.951]. (3) All cases of HRHS fetus successfully underwent FPV. The average gestational age was (27.3±0.8) weeks. The average operation time was (23.2±1.0) minutes. The ratio of tricuspid to mitral annulus ( t =-2.513, P =0.022) and the ratio of right to left ventricular length ( t =-3.373, P =0.003) were significantly improved at 6 weeks postoperatively. Ten fetuses were delivered, and there was no death after early intervention. (4) Of 13 pregnant women, 3 cases were nausea and vomiting on the day of FPV operation, the treatment of the symptoms was improved by tropisetron; one case had tolerable abdominal pain and improved without special treatment. Pregnant women had no major complications such as cardiac failure, abortion and death. (5) Chromosome karyotype analysis and microarray analysis of amniotic fluid was retained during the operation. No typical chromosome abnormality or other abnormal genetic diagnosis was found. Conclusions: FPV colud be used as an effective intervention measure to promote the development of right ventricle in HRHS fetuses. The scientific management of multidisciplinary professional technical team in perioperative period is particularly important to ensure the success of FPV and the safety of pregnant women and fetuses.

Bicuspid aortic valve (BAV) is the most common congenital heart defect, found in up to 2% of the population and associated with a 30% lifetime risk of complications. BAV is inherited as an autosomal dominant trait with incomplete penetrance and variable expressivity due to a complex genetic architecture that involves many interacting genes. In this review, we highlight the current state of knowledge about BAV genetics, principles and methods for BAV gene discovery, clinical applications of BAV genetics, and important future directions., Competing Interests: Declaration of Competing Interest None., (Copyright © 2020 Elsevier Inc. All rights reserved.)

There is a growing need to develop a common language when referring to a frequent and heterogeneous condition such as the congenital bicuspid aortic valve and its aortopathy. The following short manuscript serves as an introduction to a standard terminology for the bicuspid aortic valve and its aortopathy., Competing Interests: Declaration of Competing Interest None., (Copyright © 2020 Elsevier Inc. All rights reserved.)

We describe a case of a low birth weight neonate who presented on second day of life with progressive cyanosis and oxygen saturation of 60% by pulse oximetry. The echocardiography examination revealed a large tumor-like mass connected to the tricuspid valve, with severe obstruction of the right ventricular inflow and massive right-to-left shunt through the distended foramen ovale. A large vegetation-like lesion with calcifications was discovered intraoperatively and was debrided by shave excision technique under deep hypothermic circulatory arrest. Follow-up showed normal function of the tricuspid valve and preserved biventricular function.

We describe herein a 65-year-old woman who underwent resection of a dilated (5.1 cm) ascending aorta associated with a normally functioning congenitally bicuspid aortic valve. The patient provided the framework to discuss proper management-operative versus nonoperative-of the dilated ascending aorta associated with a normally functioning bicuspid aortic valve. Unfortunately, there is inadequate data to provide an unequivocal answer to this dilemma. Operative intervention requires that the short-term risk of the prophylactic procedure be considerably lower than the long-term risk of aortic dissection/rupture without operative intervention. Because there is no proof that operative intervention provides less morbidity and lower mortality, nonoperative management at this time seems to be the better approach., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Background: Mitral annular disjunction (MAD) is a structural abnormality where there is a separation between the mitral valve annulus and the left atrial wall which is not well understood., Methods: We conducted a systematic review to evaluate the prevalence of MAD, factors associated with MAD and clinical outcomes among patients with MAD., Results: A total of 19 studies were included in this review, and the number of noncase report studies had between 23 and 1439 patients. The pooled rate of MAD in studies of myxomatous mitral valve patients was 66/130 (50.8%, 3 studies), and among patients with mitral valve prolapse was 95/291 (32.6%, 3 studies). One study suggests that 78% of patients with MAD had mitral valve prolapse, and another suggested it was strongly associated with myxomatous mitral valve disease (HR 5.04 95% CI 1.66-15.31). In terms of clinical significance, it has been reported that MAD with disjunction > 8.5 mm was associated with nonsustained ventricular tachycardia (OR 10 95% CI 1.28-78.1). There is also evidence that gadolinium enhancement in papillary muscle (OR 4.09 95% CI 1.28-13.05) and longitudinal MAD distance in posterolateral wall (OR 1.16 95% CI 1.02-1.33) was predictive of ventricular arrhythmia and late gadolinium enhancement in anterolateral papillary muscle was strongly associated with serious arrhythmic event (OR 7.35 95% CI 1.15-47.02)., Conclusions: Mitral annular disjunction appears to be common in myxomatous mitral valve disease and mitral valve prolapse which can be detected on cardiac imaging and may be important because of its association with ventricular arrhythmias and sudden cardiac death., (© 2019 Wiley Periodicals, Inc.)

Objective: The Ross procedure has been cited as the procedure of choice for young patients requiring aortic valve replacement. However, potential for reintervention requirement in both left and right ventricular outflow tracts can be a source of concern. The aim of the present study was to describe our experience with this procedure., Methods: A retrospective chart review of all the patients who underwent the Ross procedure in a single institution was performed. National death registry records were used for late mortality., Results: Eighteen Ross procedures between May 2003 and May 2018 were performed. The median age of the cohort was 15 [interquartile range (IQR): 12-18] years. The pulmonic conduit was a homograft in 11 patients, Labcor in 5 patients, Contegra in 1 patient, and Medtronic Freestyle Valve in 1 patient. There were three early deaths. The median follow-up of 15 hospital survivors was 11 (IQR: 3-14) years. Any late mortality was not observed. In the two surviving patients with infective endocarditis, there was no recurrent infective endocarditis. Freedom from reintervention was 80% at 8 years and onward. Any risk factors associated with reintervention could not be identified. However, freedom from autograft dilatation at 10 years was 45%., Conclusion: Autograft failure is a potential problem in the long-term follow-up of Ross patients. Freedom from reintervention was satisfactory, and the type of pulmonic conduit did not affect the mid-term outcomes. In patients with infective endocarditis, the Ross procedure has a low recurrence rate, but it might have an increased risk of mortality.

A newborn with prenatally diagnosed dysplasia of both atrioventricular valves presented after birth with signs and symptoms of low cardiac output, severe regurgitation of both mitral and tricuspid valves. This combination is as rare as challenging, since it regards both the timing and management of this complex cardiac malformation. We report an early surgical repair of both atrioventricular valves in a symptomatic newborn, which improved his clinical status and, so far, delayed valve replacement.

Patients with dysfunctional right ventricular outflow tracks comprise a large portion of patients with severe congenital heart disease. Transcatheter pulmonary valve replacement in patients with dysfunctional right ventricular outflow tracks is feasible, safe, and efficacious. This article reviews current transcatheter valve replacement technology for dysfunctional right ventricular outflow tract and pulmonary valvular disease and its applications to patients with congenital heart disease. Discussed are the approach and preprocedural planning, current options, and applications of transcatheter pulmonary valve therapy. Also considered are future directions in this field as the technologies begin to develop further., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Few reports have appeared describing patients with a purely regurgitant congenitally bicuspid aortic valve (BAV) unassociated with active or healed infective endocarditis or with acute or healed aortic dissection. This report describes a large group of such patients who had replacement of the purely regurgitant BAV with or without concomitant resection of the ascending aorta. Operatively excised purely regurgitant BAVs were examined and then their clinical records were examined to confirm that the valves indeed were purely regurgitant. The patients were aged 21 to 86 years (median 50). Of the 133 patients, 114 (86%) were men. The degree of aortic regurgitation (AR) ranged from 1+ to 4+/4+. Of the 133 patients, 52 (39%) had simultaneous resection of the ascending aorta, its frequency varying inversely with the degree of AR. Histologic study of sections of the operatively excised aortas disclosed that 28 (54%) had a normal or nearly normal aorta (0-1+ loss of medial elastic fibers) and that 24 (46%) had an abnormal loss (grade 2+ -4+/4+). In conclusion, the congenitally BAV, unassociated with either infective endocarditis or aortic dissection, is a common cause of pure AR in adults in the Western World undergoing AVR for AR. About half the patients had a dilated ascending aorta and those resected were histologically abnormal half the time. Why one BAV becomes stenotic, another purely regurgitant, another the site of infective endocarditis, and another functions normally for an entire lifetime remains unclear., (Copyright © 2018. Published by Elsevier Inc.)

An 82-year-old man was found dead on the road near his home with unwitnessed interval of 3 h from final witness. He had been diagnosed with hypertension and mild aortic stenosis (AS) 13 years before death, and was continuously followed up with medication. Although a recent medical check-up related to cardiac function was stable and consistent with moderate AS, he sometimes complained of general fatigue, anorexia associated with intermittent mild fever and rare vomiting in the weeks before death. At autopsy, no lethal injury or drug intoxication was found, but congenital bicuspid aortic valve (BAV) with central rache was found. Although calcification was found in a restricted area of one cusp, valvular structural deformity was clearly milder than in typical severe AS cases. Moderate left ventricular hypertrophy without coronary disease was found. A brownish-red, soft nodular lesion was found in both adrenal glands, but no other tumorous focus was evident in any other organs. Immunohistochemical examination showed that B-lymphocyte-derived markers (CD20, melanoma associated antigen (mutated) 1, and CD79a) were exclusively positive. Therefore, we diagnosed primary adrenal lymphoma (PAL), diffuse large B-cell lymphoma phenotype. We concluded that the cause of sudden unexpected death (SUD) was adrenal insufficiency associated with PAL, with a background of moderate AS related to BAV., (Copyright © 2018 Elsevier B.V. All rights reserved.)

Competing Interests: Competing interests: None declared.

Bicuspid aortic valve, one of the most common congenital cardiac malformations, is associated with other valvular abnormalities and aortopathies. It increases the risk of thoracic aortic aneurysm and dissection, infective endocarditis, and sudden cardiac death. Patients require regular surveillance for progressive valvular disease and aortopathy. Although bicuspid aortic valve may occur sporadically, many cases are inherited, so first-degree relatives should be evaluated and genetic counseling considered., (Copyright © 2018 Cleveland Clinic.)

Congenital bicuspid aortic valve (BAV), the most common form of birth defect in humans, is associated with substantial morbidity and mortality. Increasing evidence demonstrates that genetic risk factors play a key role in the pathogenesis of BAV. However, BAV is a genetically heterogeneous disease and the genetic determinants underpinning BAV in an overwhelming majority of patients remain unknown. In the present study, the coding exons and flanking introns of the GATA6 gene, which encodes a zinc-finger transcription factor essential for the normal development of the aortic valves, were sequenced in 152 unrelated patients with congenital BAV. The available relatives of a proband harboring an identified GATA6 mutation and 200 unrelated, ethnically matched healthy individuals used as controls were also genotyped for GATA6. The functional characteristics of the mutation were analyzed by using a dual-luciferase reporter assay system. As a result, a novel heterozygous GATA6 mutation, p.E386X, was identified in a family with BAV transmitted in an autosomal dominant mode. The nonsense mutation was absent in 400 control chromosomes. Biological assays revealed that the mutant GATA6 protein had no transcriptional activity compared with its wild-type counterpart. Furthermore, the mutation disrupted the synergistic transcriptional activation between GATA6 and GATA4, another transcription factor causally linked to BAV. In conclusion, this study firstly associates GATA6 loss-of-function mutation with enhanced susceptibility to familial BAV, which provides novel insight into the molecular mechanism of BAV, implying potential implications for genetic counseling and personalized management of BAV patients., (Copyright © 2018 Elsevier B.V. All rights reserved.)

Background: Unicuspid unicommissural aortic valve is an extremely rare congenital anomaly that usually presents in adulthood but can rarely present in infancy. We report a 17-year-old patient with congenital aortic stenosis secondary to unicuspid unicommissural aortic valve that was successfully treated with aortic valve replacement., Case Presentation: The patient was diagnosed with aortic stenosis after a murmur was heard in the newborn nursery and subsequently underwent aortic balloon valvuloplasty 6 weeks after birth. He had been regularly followed up since and underwent numerous cardiac catheterizations, including another aortic balloon valvuloplasty at age 13. During follow-up at age 17, the patient presented with symptomatic severe aortic stenosis and mild left ventricular hypertrophy. Aortic valve replacement was planned since the patient was nearly adult-sized and to reduce the risk of cardiac decompensation. During the operation an unicuspid unicommissural aortic valve was revealed. The patient recovered well post-operatively. He was discharged 5 days after the surgery in good condition and was completely symptom-free at follow-up 6 weeks later., Conclusions: Unicuspid aortic valve is a rare congenital anomaly that can cause congenital aortic stenosis. It is seldom diagnosed pre-operatively but should be suspected in infants presenting with aortic stenosis.

We demonstrate the incremental value of live/real time three-dimensional transthoracic echocardiography (3DTTE) over the two-dimensional modality in the identification of all three cusps of the pulmonary valve in patients in whom only two leaflets could be detected by the latter technique. This was because of the ability of 3DTTE to view the cusps enface permitting assessment of relationship to one another and the surrounding cardiac structures. In addition, 3DTTE showed not only the potential errors that can occur in pulmonary valve cusp identification when the two-dimensional modality is used alone but also how some of these errors can be avoided by paying attention to the surrounding structures., (© 2018 Wiley Periodicals, Inc.)

Infective endocarditis (IE) is uncommon but a very serious infection during pregnancy. In most cases, the disease tends to run a subacute course and involves the mitral valve. We present the case of a 25-year old pregnant female who developed shortness of breath and fever 2 weeks prior to parturition. The symptoms did not subside after her delivery, which was carried out via c-section. Based on vegetations attached to aortic valve cusps and positive blood culture for staphylococcus Aureus, the diagnosis of infective endocarditis was made.

Accessory mitral valve tissue (AMVT) causing left ventricular outflow tract obstruction (LVOTO) is rare. We report a case of AMVT causing severe LVOTO resulting in acutely progressive symptoms of near-collapse. Urgent surgical resection eliminated the patient's life-threatening symptoms. AMVT should be considered among potential LVOTO diagnoses, and early surgical intervention may be required.

Bicuspid aortic valve (BAV) is the most common congenital heart defect. BAV is associated with aortic stenosis and insufficiency, and aortic dilation in adult groups, but data in pediatric groups are limited. We sought to assess the impact of BAV morphology on aortic valve disease and aortic dilation in pediatric patients. We performed a retrospective review of all echocardiograms in patients with isolated BAV who were followed at our institution from July 2002 to July 2012. BAV morphology, aortic valve stenosis and/or insufficiency, and aortic dimensions were measured manually. Comparisons were made between right-left cusp fusion (RL) and right-noncoronary cusp fusion (RN) BAV morphologies. Generalized least square models were fit to analyze the impact of specific variables on aortic dilation. There were 1075 echocardiograms in 366 patients (72% male) with isolated BAV. Aortic valve insufficiency and stenosis were more common in RN (p < 0.001 for both). The median aortic sinus Z score was higher in the RL (0.47; IQR - 0.31 to 1.44) than in the RN group (0.02; - 0.83 to 0.82) (p < 0.001). There was no difference in median ascending aorta Z score between groups. Patients with the highest weights had larger aortas (p < 0.001), but the absolute difference between the highest and lowest weight groups was small (1.5 mm). The impact of BAV morphology on aortic valve disease and aortic dilation in pediatric patients presages that seen in adults. Patient body weight does not make significant clinical impacts on aortic diameters, suggesting that Z scores for aortic diameters should be based on ideal body weights.

Aggregating evidence suggests that genetic determinants play a pivotal role in the pathogenesis of the congenitally bicuspid aortic valve (BAV). BAV is of pronounced genetic heterogeneity, and the genetic components underlying BAV in an overwhelming majority of patients remain elusive. In the current study, the whole coding exons and adjacent introns, as well as 5' and 3' untranslated regions of the GATA4 gene, which codes for a zinc-finger transcription factor crucial for the normal development of the aortic valve, were screened by direct sequencing in 150 index patients with congenital BAV. The available family members of an identified mutation carrier and 300 unrelated, ethnically matched healthy individuals used as controls were also genotyped for GATA4. The functional effect of the mutation was characterized using a dual-luciferase reporter assay system. As a result, a novel heterozygous GATA4 mutation, p.E147X, was identified in a family with BAV transmitted in an autosomal dominant pattern. The nonsense mutation was absent in 600 control chromosomes. Functional deciphers revealed that the mutant GATA4 protein lost transcriptional activity compared with its wild-type counterpart. Furthermore, the mutation disrupted the synergistic transcriptional activation between GATA4 and NKX2.5, another transcription factor responsible for BAV. In conclusion, this study associates the GATA4 loss-of-function mutation with enhanced susceptibility to a BAV, thus providing novel insight into the molecular mechanism underpinning the BAV., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Congenital double-orifice mitral valve (DOMV) is a rare malformation that may affect both mitral valve and apparatus, and it is usually associated with other cardiac disorders. Asymptomatic DOMV is extremely rare. We present two similar cases of asymptomatic congenital double-orifice mitral valve. Both cases presented functionally normal mitral valve apparatus with two equally sized orifices, which were formed by a central fibrosis. In addition, final confirmatory diagnosis was made using three-dimensional (3D) echocardiography in both cases.

Repeat mitral valve replacement in patients who underwent mechanical replacement during infancy may be technically very difficult due to the small-sized annulus accommodating the prosthesis. That can lead to dense fibrosis of the small annulus onto the sewing cuff. An attempt at explanting the prosthesis carries high risk of circumflex coronary artery injury or atrioventricular junction disruption. We present a novel approach to prevent a disaster in such a difficult scenario-implanting the new prosthesis above the previous prosthetic housing after removing the prosthetic leaflets., (Copyright © 2018 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

In 2013, we published a simple method for aortic valve reconstruction in children using fixed pericardium in this journal. This was not expected to be a durable solution but a temporizing measure in the absence of other solutions, in order to buy time for growth that would allow subsequent prosthetic valve insertion. As such, this was implemented in 3 patients with excellent immediate results, as reported in our original publication. We are now writing to provide longer term information. In 2 cases, a newborn and a 12-year-old boy, the valve calcified heavily at 4 years' follow-up. By then, the valvar annulus had grown sufficiently to accept a prosthetic valve in both cases. In these 2 cases, the material used for valve reconstruction was autologous pericardium fixed in 0.6% gluteraldehyde. In the third patient, a 3-year-old girl, the valve failed by dehiscence of the suture line 6 months following surgery. In this case, fixed bovine pericardium had been used, which is more rigid than autologous pericardium, thus possibly explaining the dehiscence. However, the pericardial patch was not calcified. Thus, the described method lived up to our expectation of buying time for growth for later insertion of a prosthetic valve in 2 of the 3 cases., (© The Author 2017. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Background: Unicuspid aortic valve (UAV) is a rare disorder, often difficult to distinguish from bicuspid aortic valve (BAV). BAV and UAV share valve pathology such as the presence of a raphe, leaflet fusion, aortic stenosis, aortic regurgitation, and/or ascending aortic dilatation, but a comprehensive echocardiographic comparison of patients with UAV and BAV has not been previously performed., Methods: We investigated UAV and BAV patients at an early stage of disease included in GenTAC, a national registry of genetically related aortic aneurysms and associated cardiac conditions. Clinical and echocardiographic data from the GenTAC Registry were compared between 17 patients with UAV and 17 matched-controls with BAV., Results: Baseline characteristics including demographics, clinical findings including family history of BAV and aortic aneurysm/coarctation, and echocardiographic variables were similar between BAV and UAV patients; aortic stenosis was more common and more severe in patients with UAV. This was evidenced by higher mean and peak gradient, smaller aortic valve area, and more advanced valvular degeneration (all P < .05). There were no significant differences in aortic dimensions, with a similar pattern of enlargement of the ascending aorta., Conclusions: The similar baseline characteristics with more accelerated aortic valve degeneration and stenosis suggest that UAV represents an extreme in the spectrum of BAV syndromes. Therefore, it is reasonable to consider application of recommendations for the management of patients with BAV to those with the rarer UAV., (© 2017 Wiley Periodicals, Inc.)

Objectives: To determine a clinical standard for post-repair common atrioventricular valve orifice area based on mid- to long-term valve function in patients with a functional single ventricle., Methods: The medical records of 19 single-ventricle patients who underwent common atrioventricular valve plasty from July 1988 to January 2013 were retrospectively reviewed. Bivalvation valvuloplasty was performed in 7 patients with relatively hypoplastic leaflets. The relationship between the orifice area of the repaired common atrioventricular valve measured intraoperatively and valve function and ventricular volume in the early postoperative period (median, 9.5 months) and at mid- to long-term follow-up (median, 4 years) were analysed., Results: Post-repair valve area was significantly positively correlated with valve regurgitation severity in the early postoperative period (P = 0.001, r = 0.69) and at mid- to long-term follow-up (P = 0.02, r = 0.57). Patients who did not undergo bivalvation had favourable valve function at mid- to long-term follow-up and in the early postoperative period when the post-repair valve area was 96-136% of the normal mitral valve area. Bivalvation patients had significantly more valve regurgitation in the early postoperative period than patients without bivalvation, despite equivalent repaired valve area (P = 0.02)., Conclusions: The post-repair orifice area of the common atrioventricular valve is significantly related to postoperative valve function. The clinical standard of post-repair valve orifice area might be 96-136% of the normal mitral valve area in patients undergoing repair without bivalvation. Patients undergoing bivalvation require greater reduction to obtain favourable mid- to long-term valve function., (© The Author 2017. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Objectives: Tetralogy of Fallot with absent pulmonary valve syndrome is a rare form of tetralogy of Fallot with dilatation of large pulmonary arteries. Prognosis is related to the severity of the cardiac malformation and to bronchial tree compression by dilated pulmonary arteries. This study analyses the prenatal echographic lung appearance in fetuses with tetralogy of Fallot with absent pulmonary valve and discusses its significance., Methods: We carried out a retrospective review of fetal and postnatal files of nine fetuses diagnosed with tetralogy of Fallot with absent pulmonary valve syndrome in our institution. Correlations of prenatal ultrasound and cardiac imaging findings were obtained with outcome., Results: Abnormal heterogeneous fetal lung echogenicity was detected in eight cases out of nine, always associated with significant lobar arterial dilatation. This aspect was well correlated with postnatal imaging and outcome in the four neonatal cases. The only fetus with normal lung echogenicity also had lower degree of pulmonary artery dilatation in the series., Conclusions: This study demonstrates that a heterogeneous ultrasound appearance of the fetal lungs can be detected in utero in the most severe cases. This aspect suggests an already significant compression of the fetal bronchial tree by the dilated arteries that may have prognostic implications.

Objectives: To study the recent trends and outcomes of congenital mitral valve surgery in children., Methods: From 2008 to 2014, 84 procedures in 66 consecutive patients (41 procedures in 31 patients with mitral stenosis and 43 procedures in 35 patients with mitral regurgitation) were retrospectively evaluated. The mean age at surgery was 4.3 ± 5.4 years, and 27 patients (41%) were neonates or infants., Results: Seven (11%) patients died during the follow-up period of 3.2 ± 2.3 years and 5 (71%) were <1 year. Ten mitral valve replacements were performed in 8 patients, including 1 pulmonary valve homograft, 3 Contegra conduits of 12 mm thickness in the intra-annular position and 6 mechanical valves. Shone's syndrome, dysplastic valve, a need for valve replacement and age <1 year were the risk factors for death or reoperation in a univariable analysis, while in a multivariable analysis of all patients, valve replacement and age <1 year remained as risk factors. In a multivariable analysis of 27 patients aged <1 year, mitral valve dysplasia was a significant risk factor for reoperation or death. The 5-year rate of freedom from death or reoperation in neonates or infants was 55% and that in patients aged >1 year was 88% (P = 0.003)., Conclusions: An age of <1 year, mitral valve dysplasia and a need for mitral valve replacement were associated with a higher incidence of death or reoperation. Primary mitral valve replacement or univentricular strategy may have to be considered for symptomatic neonates with Shone's syndrome., (© The Author 2017. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

This study sought to evaluate the prognostic value of left ventricular (LV) fibrosis assessed by late gadolinium enhancement (LGE) of the myocardium during cardiac magnetic resonance (CMR) imaging in patients with bicuspid aortic valve (BAV), which is associated with early aortic valve fibrosis and calcification. To what degree the LV myocardial wall is affected by fibrosis and its prognostic value is currently unknown. This is a retrospective, single-center study evaluating all adult patients with BAV who had CMR and followed from March 2002 to March 2016. CMR and transthoracic echocardiogram images were reviewed. Clinical data were abstracted from the electronic medical record. A total of 29 patients were included in the study, of which 11 (38%) had CMR studies that demonstrated the presence of LGE. Patients with LGE had significantly higher aortic valve mean gradients by echocardiography when compared with LGE-negative patients (30.3 ± 7.2 mm Hg vs 14.7 ± 3.6 mm Hg, p = 0.049). They were also more likely to have LV hypertrophy. Patients with LGE were 10 times more likely to need aortic valve replacement within 1 year of the CMR study than did patients without LGE (55% vs 5.5%, p = 0.0028). In conclusion, evaluation of LGE by CMR as a marker of LV myocardial fibrosis can have additional prognostic value when evaluating patients with aortic stenosis secondary to BAV., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Objectives: Any aortic valve (AoV) operation in children (repair, Ross or mechanical replacement) is a palliation and reinterventions are frequent. AoV repair is a temporary solution primarily aimed at allowing the patient to grow to an age when more definitive solutions are available. We retrospectively analysed AoV repair effectiveness across the whole age spectrum of children, excluding neonates and AoV disease secondary to congenital heart disease., Methods: From 2003 to 2015, 193 consecutive patients were included. The mean age was 9.2 ± 6.9 years (22% <1 year); 86 (45%) had a preceding balloon valvuloplasty. The indications for the procedure were stenotic (n = 123; 64%), regurgitant (n = 63; 33%) or combined (n = 7; 4%) disease. The procedures performed were commissurotomy shaving (n = 74; 38%), leaflet replacement (n = 78; 40%), leaflet extension (n = 21; 11%) and neocommissure creation (n = 21; 11%). Post-repair geometry was tricuspid in 137 (71%) patients., Results: The 10-year survival rate was 97.1%. Freedom from reoperation and replacement at 7 years was, respectively, 57% (95% confidence interval, 47-66) and 68% (95% confidence interval, 59-76). In multivariate analysis, balloon dilatation before 6 months, the absence of a developed commissure, a non-tricuspid post-repair geometry and cross-clamp duration were predictors for reoperation and replacement. After a mean follow-up period of 5.1 ± 3.0 years, 145 (75%) patients had a preserved native valve, with undisturbed valve function (peak gradient <40 mmHg, regurgitation ≤mild) in 113 (58%)., Conclusions: Aortic valve repair in children is safe and effective in delaying the timing for more definitive solution. Surgical strategy should be individualized according to the age of the patient. Avoidance of early balloon dilatation and aiming for a tricuspid post-repair arrangement may improve outcomes., (© The Author 2017. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Purpose of Review: The number of pregnancies complicated by valvular heart disease is increasing. This review describes the hemodynamic effects of clinically important valvular abnormalities during pregnancy and reviews current guideline-driven management strategies., Recent Findings: Valvular heart disease in women of childbearing age is most commonly caused by congenital abnormalities and rheumatic heart disease. Regurgitant lesions are well tolerated, while stenotic lesions are associated with a higher risk of pregnancy-related complications. Management of symptomatic disease during pregnancy is primarily medical, with percutaneous interventions considered for refractory symptoms. Most guidelines addressing the management of valvular heart disease during pregnancy are based on case reports and observational studies. Additional investigation is required to further advance the care of this growing patient population.

Objectives: The Technical Performance Score (TPS) has been shown to be predictive of postoperative mortality, morbidities and reinterventions following various cardiac procedures in children. We hypothesized that TPS is also a predictor of mitral valve repair outcomes., Methods: A review of patients who underwent mitral valve repair from January 2000 to December 2013 was performed. Primary repair of complete atrioventricular defect was excluded. The scores were determined according to previously published criteria based on the need for reintervention and predischarge echocardiograms: Class 1 (no residua), Class 2 (minor residua) or Class 3 (pacemaker implantation, major residua or reintervention for major residua prior to discharge). Cox proportional hazard models and Kaplan-Meier estimator were used., Results: A total of 587 patients underwent mitral repair (median age 2.6 years). Median follow-up duration was 3 years. There were 125 (21.3%) post-discharge mitral reinterventions and freedom from reintervention was 85.2%, 78.2% and 69.4% at 1, 2 and 5 years, respectively. Both TPS Class 2 [hazard ratio (HR) 3.6, 95% confidence interval (CI) 1.4-10.0; P  = 0.02] and Class 3 (HR 8.7, 95% CI 3.0-25.1; P  < 0.001) were associated with post-discharge reinterventions. There were 31 late deaths/transplantations, and transplant-free survival at 1, 2 and 5 years was 97.8%, 95.3% and 93.2%. TPS 3 was associated with decreased post-discharge transplant-free survival (HR 5.5, 95% CI 1.2-25.0; P  = 0.03). Post-discharge mitral reintervention was not associated with increased mortality., Conclusions: The TPS is a strong predictor of midterm mortality and post-discharge mitral reintervention in congenital patients who underwent mitral repair., (© The Author 2017. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Objective: Until now, no study established a morphometric evaluation of the truncal valve dysplasia and a description of its different presentation patterns. Thus, authors conducted an anatomopathological study describing the gross features and histological findings of the truncal valve., Methods: 50 common arterial trunk (CAT) specimens were examined. The number of valvar leaflets was determined and valvar dysplasia was classified as absent, mild, moderate or severe. Selected leaflets were sectioned and submitted to histological analysis and linear measurements (thickness, length and area), besides quantification of collagen area fraction., Results: 28 (56%) valves presented three, 15 (30%) four and 7 (14%) two leaflets. Valvar dysplasia was absent in 13 (26%) cases, mild in 19 (38%), moderate in 6 (12%) and severe in 12 (24%). A significant association was found between the presence of four leaflets and valvar dysplasia (p<0.001). Single coronary ostium was more common in two-leaflet cases than in three-leaflet cases (p=0.037). Leaflets medial thirds were thicker in the more dysplastic valves (p=0.006) and in those presenting anarchic collagen distribution (p=0.002)., Conclusions: CAT semilunar valves present two main patterns. The first characterised by three leaflets and absent or mild dysplasia and the second by four leaflets and severe dysplasia. Still, great variability regarding thickness, microscopic organisation of the extracellular matrix and proportions of leaflets' dimensions exists, which may impact on the surgical outcomes., Competing Interests: Competing interests: None declared., (Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/.)

We report a case of successful heterograft aortic valve replacement following an impede Ross procedure in a 48-year-old man presenting with a congenital bicuspid pulmonary valve. The patient was admitted for aortic valve stenosis that required an aortic valve replacement (AVR). Owing to his young age and reluctance to long-term anticoagulation therapy, it was decided to do an AVR by pulmonary autograft. During surgery, the anatomical unsuitability of the graft was discovered leading to the procedure's readjustment.