A rare combination of cardiovascular anomaly: Aortic stenosis at sinotubular junction level and discontinuity of right coronary artery.

BACKGROUND: Supravalvular aortic stenosis (SVAS) and congenital discontinuity of right coronary artery are both rare congenital cardiovascular abnormalities. This is the first case report about SVAS that occurred with the congenital discontinuity of right coronary artery. MATERIALS & METHODS: A 3-month-old female infant presented with aortic stenosis at sinotubular level and congenital right coronary artery deficiency. According to cardiovascular CT results, Doty technique was adopted to restore the aortic root geometry under cardiopulmonary bypass. An angioplasty was performed to establish right coronary blood flow at the same time. The patient had no abnormal cardiac symptoms after surgery. The postoperative echocardiogram revealed a normal laminar flow of the right coronary artery into the right coronary sinus, normal aortic blood flow and normal myocardial functions. DISCUSSION: SVAS is characterized by the stenosis of the lumen of the ascending aorta above the aortic valve. Congenital discontinuity of RCA is probably related to dysplasia or congenital occlusion of the RCA during the development of embryo. This kind of malformation may lead to the deficiency of blood supply in sinoatrial and atrioventricular node, eventually causing their dysfunction, which usually leads to arrhythmias as the main manifestations. Angioplasty can improve blood supply of the heart without increasing the risk of major complications, and perioperative prognosis revealed good. This case image also suggested that cardiovascular CT can provide excellent visualization of complex vascular anatomies. CONCLUSIONS: We reported this rare combination of malformations consisted of SVAS and discontinuity of right coronary artery. We treated this patient with the Doty technique and angioplasty procedures.

Coarctation of aorta combined with multiple aneurysms.

We reported a case of a 53-year-old patient with coarctation of the aorta and multiple aneurysmatic changes on the aortic arch. Enhanced computed tomography and reconstruction revealed significant coarctation and multiple aneurysmatic dilatations. The patient underwent stent implantation and was discharged with symptoms relieved. Follow-up examination progression of aneurysms, however, without symptoms.

Transannular patch repair of tetralogy of Fallot with or without monocusp valve reconstruction: a meta-analysis.

BACKGROUND: Tetralogy of Fallot (TOF) is one of the most common cyanotic congenital heart diseases. Pulmonary regurgitation is the most common and severe comorbidity after transannular patch (TAP) repair of TOF patients. It has not been confirmed whether a TAP repair with monocusp valve reconstruction would benefit TOF patients in perioperative period compared to those without monocusp valve reconstruction. The purpose of the study is to review and analyze all clinical studies that have compared perioperative outcomes of TOF patients undergoing TAP repair with or without monocusp valve reconstruction and conduct a preferable surgery. METHODS: Eligible studies were identified by searching the electronic databases. The year of publication of studies was restricted from 2000 till present. The primary outcome was perioperative mortality, and secondary outcomes included cardiopulmonary bypass time, aortic cross-clamp time, ventilation duration, ICU length of stay, hospital length of stay, perioperative right ventricular outflow tract (RVOT) pressure gradient, and moderate or severe pulmonary regurgitation (PR). The meta-analysis and forest plots were drawn using Review Manager 5.3. Statistically significant was considered when p-value ≤ 0.05. RESULTS: Eight studies were included which consisted of 8 retrospective cohort study and 2 randomized controlled trial. The 10 studies formed a pool of 526 TOF patients in total, in which are 300 undergoing TAP repair with monocusp valve reconstruction (monocusp group) compared to 226 undergoing TAP repair without monocusp valve reconstruction (non-monocusp group). It demonstrated no significant differences between two groups in perioperative mortality (OR = 0.69, 95% CI 0.20-2.41, p = 0.58). It demonstrated significant differences in perioperative cardiopulmonary bypass time (minute, 95% CI 17.93-28.42, p < 0.00001), mean length of ICU stay (day, 95% CI - 2.11-0.76, p < 0.0001), and the degree of perioperative PR (OR = 0.03, 95% CI 0.010.12, p < 0.00001). Significant differences were not found in other secondary outcomes. CONCLUSION: Transannular patch repair with monocusp valve reconstruction have significant advantages on decreasing length of ICU stay and reducing degree of PR for TOF patients. Large, multicenter, randomized, prospective studies which focuse on perioperative outcomes and postoperative differences based on long-term follow-up between TAP repair with and without monocusp valve reconstruction are needed.

Huge right ventricle cardiac fibroma in a child patient.

In this case, we describe a 9-year-old child who presented with chest pain due to a huge right ventricle fibroma. With the support of cardiopulmonary bypass, the tumor was successfully removed, but to avoid right ventricular rupture and tricuspid valve injury, only a small amount of tumor tissue remained in the myocardial tissue. Pathological examination confirmed that the ventricular tumor was a fibrous borderline tumor. The patient has normal cardiac and tricuspid function at a 3-month follow-up. It is reported that the prognosis of patients younger than 17 years old at the time of diagnosis is poor, and the long-term prognosis of this child needs further follow-up.

A large aortopulmonary window found in the setting of a right aortic arch with discontinuous pulmonary arteries.

Aortopulmonary window (APW) is a rare but serious congenital cardiac malformation, most patients with APW will die from congestive heart failure within one year after birth. In fact, patients with large APW is rarely seen in childhood or adult life. However, we report an older child with a large anomalous "window" on the ascending aorta, with discontinuous pulmonary arteries, and the left pulmonary artery (LPA) arising via a left-sided arterial duct in the presence of a right aortic arch. Preoperative diagnosis made by echocardiography and chest computerized tomography revealed anatomical futures clearly. Cardiac catheterization indicated that the pulmonary resistances indices were 2.92 Wood unit/m2 in LPA and 3.35 Wood unit/m2 in RPA, Q p : Q s was 3.26. This patient underwent surgical correction at the age of 10 and successfully survived.

Anomalous Right Coronary Artery Arising from the Pulmonary Artery with Associated Enlargement of the Left Coronary Artery: A Case Report.

An anomalous right coronary artery arising from the pulmonary artery (ARCAPA) is among the least common form of congenital coronary anomalies, accounted for the incidence of only 0.002% in the general population. Most ARCAPA patients have no symptoms but may develop myocardial ischemia. Surgical correction of the anomaly is recommended to prevent subsequent fatal outcomes. Here, we reported a case of a 2-year-old female child initially hospitalized for diarrhea, but later diagnosed with an ARCAPA through echocardiogram and computed tomography. Surgical reimplantation of the right coronary artery from the pulmonary artery to the ascending aorta was performed. The patient recovered well from the surgery with no postoperative complications. In the follow-up assessments, normal coronary function and myocardial effusion were demonstrated.

Tetralogy of fallot in addition to anomalous aortic origin of a coronary artery in a 1-year-old boy: a case report.

BACKGROUND: Anomalous aortic origin of a coronary artery (AAOCA) is a rare congenital heart disease, characterized by the coronary artery inappropriately originates from the aorta. It is usually classified according to the sinus where the coronary artery arises from, while anomalous origin of the right coronary being the most common type. CASE PRESENTATION: In this case report, we described a rare case of Tetralogy of Fallot (TOF) in a 1-year-old boy, who also had the anomalous right coronary artery that originated from the left coronary sinus without an intramural segment. Besides TOF repair, lateral pulmonary translocation was undertaken in order to avoid risks of myocardial ischemia. CONCLUSION: We successfully completed a one-stage operation consisting of TOF repair and pulmonary artery translocation in a 1-year-old boy. We advocated early operation of pulmonic translocation for AAOCA patients without an intramural segment instead of unroofing procedure.

Tetralogy of Fallot with Double Aortic Arch in an 8-Month-Old Girl: A Rare Association.

Double aortic arch (DAA) is an extremely rare congenital anomaly that can be divided into right dominant, left dominant, and balanced DAA according to the relative size of the two arches. The incidence of balanced DAA is only 5% among double arch anomalies. DAA is symptomatic only when it produces symptoms secondary to compression of the trachea or esophagus. DAA is rarely associated with other congenital heart diseases. In this report, we present a rare case of asymptomatic DAA combined with Tetralogy of Fallot (TOF) in an 8-month-old girl.

Anomalous aortic origin of the coronary arteries in a 12-year-old male: a case report.

BACKGROUND: Anomalous aortic origin of the coronary artery (AAOCA) is a rare congenital cardiac disease that can cause sudden cardiac death. This condition may be corrected with surgery. Among the different surgical techniques used to correct this malformation, the most common are unroofing and lateral pulmonary translocation. CASE PRESENTATION: Herein, we present a multimodal imaging approach to identifying AAOCA in a 12-year-old male. We also successfully adopted a new operative method, neo-ostium creation combined with lateral pulmonary translocation to correct AAOCA. The detailed imaging and intraoperative data has not been reported in the literature. CONCLUSIONS: Although several surgical methods exist to reverse the complications of AAOCA, we offer an innovative surgical technique that is easier, faster, and effective.

Surgical management of double outlet right ventricle with aortopulmonary window.

We report a case of double outlet right ventricle in combination with an aortopulmonary window in a 20-month-old patient and discuss its surgical management.

Modified Warden procedure for partial anomalous pulmonary venous return to the superior vena cava.

BACKGROUND: We report the long-term outcomes of our modified Warden cavoatrial technique, originally reported in 2010, for the treatment of anomalous pulmonary veins, which insert into the superior vena cava (SVC). METHODS: This study was conducted between 2007 and 2015 in 26 patients (18 females, eight males) with a mean age of 22 years (range, 4-70 years). Twenty-four patients had a sinus venosus atrial septal defect and one patient had two partial anomalous pulmonary venous connections to the SVC. Two patients with persistent left SVC underwent right SVC division without reimplantation. RESULTS: There were no short- or long-term mortalities. The mean follow-up was 4.6 years (range, three months to eight years). All patients had unobstructed caval and pulmonary venous flow and no long-term arrhythmias. CONCLUSIONS: The modified cavoatrial anastomosis technique has shown excellent results with unobstructed pulmonary and systemic venous flow without long-term arrhythmias.

A review regarding the article 'Supervised exercise training in heart failure with preserved ejection fraction: A systematic review and meta-analysis of randomized controlled trials'.

Heart failure is a clinical syndrome with different etiologies and phenotypes. For all forms, supervised exercise training and individual physical activity are class IA recommendations in current guidelines. Over the past two decades it has become recognized that physical deconditioning may play a key role in the progression of symptoms and poor outcomes. A number of prior studies have assessed the ability of exercise training to improve functional capacity in patients with HF. Most of these previous studies showed positive effects of exercise training on exercise capacity, quality of life, and biomarkers and observed relatively few complications during training. These studies also suggested that exercise training might improve survival and decrease HF hospitalizations. Despite the failure of pharmacological interventions to reduce mortality and hospitalization in HFpEF, exercise training has shown efficacy in improving maximal exercise capacity, assessed through peak oxygen consumption (peak V˙o2), in clinically stable patients. Nonetheless, there remains a safety concern regarding exercise training in HF. Although the complication rate for patients participating in cardiac rehabilitation has been reported to be extremely low, the complication rate for HF patients in clinical trials of exercise training has been substantially higher. One potential reason is the 100-fold increased risk for myocardial infarction and 50-fold increased risk of sudden death that exercisers, who are habitually sedentary, experience when initiating exercise training. Additionally, comprehensive insights into the potential effects of exercise training, not only on critical clinical outcomes such as hospitalization, mortality, and cardiovascular events but also on cost-effectiveness, require longer-term trials.

A review regarding the article 'Impact of tricuspid regurgitation severity on mortality in pulmonary hypertension patients: A comprehensive analysis.

Pulmonary hypertension (PH) presents as a complex hemodynamic and pathophysiologic state present in many cardiovascular, respiratory, and systemic diseases. PH is considered to have a higher risk of cardiovascular events and mortality. The most common type of functional tricuspid regurgitation (TR) is associated with PH. Secondary TR, resulting not from intrinsic valvular pathology but from distortion of the valve apparatus due to right ventricular remodeling and dilation, is commonly seen in the setting of PH. It has been increasingly recognized as not merely a bystander but a significant contributor to the worsening of symptoms and decline in functional status. However, the extent to which TR impacts the clinical course and mortality in PH remains a subject of active investigation. The simultaneous presence of PH and secondary tricuspid regurgitation (STR) portends particularly poor outcomes. However, not all patients with PH develop significant TR, and the mechanisms and clinical implications underlying this phenomenon remain unclear. TR is a highly prevalent echocardiographic finding in the general population. Historically considered as the "forgotten valve disease" by clinicians and interventional cardiologists, TR has become a hot topic in cardiovascular interventions over recent years. If left untreated until severe, as often occurs, TR correlates with consistent morbidity and mortality, and a variety of surgical and percutaneous treatments have therefore been proposed. Mortality from isolated surgical repair of TR remains higher than that from surgery of any other valve insufficiency and a large number of patients are often deemed not eligible for surgical repair.

A review regarding the article 'Prevalence of Valvular Heart Disease in Cardiac Amyloidosis and Impact on Survival'.

Cardiac amyloidosis (CA) is a condition characterized by the accumulation of amyloid fibrils in the heart muscle, resulting in an infiltrative cardiomyopathy. The presence of amyloid protein can impact different parts of the heart, including the valves. Limited data is available on the prevalence and prognostic significance of valvular heart disease (VHD) in CA. However, advancements in imaging technology have allowed for accurate noninvasive diagnosis of CA, eliminating the need for confirmatory endomyocardial biopsy and improving our understanding of this dual pathology. The development of targeted drug therapies for CA and transcatheter valve replacement or repair for VHD has significantly improved the prognosis for patients with both conditions. This review will discuss the findings of this original research and provide an overview of current researches on VHD in CA, as well as the progress in diagnosing and treating CA with VHD.

Precordial pain induced by the isolated cardiac hydatid cyst in interventricular septum: a case report.

BACKGROUND: Human hydatid disease occurs after infection with Echinococcus granulosus, mainly involves liver and lung, while hydatid involves heart is infrequent. A great majority of hydatid diseases could be asymptomatic, and incidentally found through examination. Here, we reported a woman who suffered an isolated cardiac hydatid cyst located at the interventricular septum. CASE PRESENTATION: A 48-year-old woman presented intermittent chest pain was admitted to the hospital. Imaging examination revealed a cyst located at the interventricular septum near the right ventricular apex. Considering medical history, radiological findings and serological results, cardiac hydatid disease was suspected. The cyst was successfully removed, while pathological biopsy confirmed the diagnosis of infection of Echinococcus granulosus. Postoperative course was uneventful, the patient was discharged from hospital without complications. CONCLUSION: For symptomatic cardiac hydatid cyst, surgical resection is necessary to avoid progression of disease. During surgical procedure, appropriate methods to reduce the potential risk of hydatid cyst metastasis are essential. Besides surgery, combined with regular drug therapy is an effective strategy to prevent reappearance.

A right ventricular diverticulum with a very special cardiac anomaly.

Cardiac diverticula are rare congenital anomalies. Among them, right ventricular diverticula are far fewer than left ventricular diverticula. Herein, we write to share an exceedingly rare case of a special right ventricular diverticulum connecting to left ventricle through a tunnel-like structure originating from the membranous ventricular septum. Surgical closure of the origin of the connecting tunnel was performed, while the right ventricular diverticulum was preserved. Postoperative recovering was uneventful.

Case report: Reconstruction of the long-gap unilateral absence of right pulmonary artery with contralateral pulmonary artery flap and autologous pericardial graft.

Unilateral absence of pulmonary artery (UAPA) is a rare type of congenital abnormality that may coexist with other congenital abnormalities or present as an isolated lesion, the latter form can be asymptomatic. Surgical procedure is usually carried out when UAPA was diagnosed with significant symptoms, and the aim of surgery is to restore the pulmonary flow distribution. The right-side UAPA is a considerable challenge for surgeons to process surgery, however, technical description of this type of UAPA are limited. Here we described a rare case of a two-month girl with absence of right pulmonary artery, we presented a technique that reconstructs this long-gap UAPA with contralateral pulmonary artery flap and autologous pericardial graft.