The abnormal origin of the left pulmonary artery (LPA) from the ascending aorta is a rare cardiac condition that is often associated with several other congenital defects. In this paper, we report the case of an infant who presented with recurrent infections and was prenatally diagnosed with tetralogy of Fallot (TOF). During echocardiography, various other cardiac defects such as ventricular septal defects (VSD), pulmonary stenosis (PS), and dilated right heart chambers were identified. Furthermore, cardiac catheterization revealed an anomalous origin of the LPA arising from the aorta associated with a narrow pulmonary annulus. Due to both conditions sharing a similar embryological course, the condition is commonly associated with a conotruncal defect known as DiGeorge syndrome. Together, the overall combination of cardiac anomalies is both unusual and unique. This case study explains the clinical associations, embryological origin, and surgical management of this condition in an infant.

Ventricular-assist device therapy for small patients with congenital heart disease is challenging due to its complex anatomy and hemodynamics. We describe a 3-year-old patient with heart failure with truncus arteriosus in the palliative stage. The patient underwent palliative right ventricular outflow tract reconstruction following bilateral pulmonary artery banding. At 6 months of age, the patient developed severe truncal valve regurgitation and left ventricular dysfunction. Emergent truncal valve replacement with a mechanical valve was performed, but left ventricular dysfunction persisted. At 3 years of age, the patient developed acute progression of heart failure triggered by influenza infection. The patient was intubated and transferred to our center to determine the indication for heart transplantation. On the second day after admission, signs of multiorgan failure appeared. Emergent ventricular-assist device implantation for both ventricles was performed with truncal valve closure, ventricular septal defect closure, atrial septal defect closure, and re-right ventricular outflow tract reconstruction. The right ventricular-assist device was successfully removed on the seventh postoperative day. Due to the small pulmonary arteries, severe pulmonary stenosis persisted after ventricular-assist device implantation, but it gradually improved with multiple pulmonary angioplasties. The patient was registered in the Japanese organ transplant network and is awaiting a donor organ in a stable condition.

UNASSIGNED: In repaired tetralogy of Fallot (ToF) patients with residual right ventricular (RV) outflow tract obstructions (RVOTO), risk stratification and timing of re-interventions are based on RVOTO gradients. However, this might be insufficient to prevent RV dysfunction. Instead, assessment of RV to pulmonary arterial (RV-PA) coupling allows integrated assessment of RV function in relationship to its afterload and could be of additional value in clinical decision-making.
UNASSIGNED: Two patients with repaired ToF and residual RVOTO without pulmonary regurgitation underwent right heart catheterization (RHC) and cardiac magnetic resonance imaging. We determined RV end-systolic elastance (Ees), arterial elastance (Ea) and RV-PA coupling (Ees/Ea) using single-beat RV pressure-volume analysis. Patient 1 was asymptomatic despite severely increased RV pressures and a left pulmonary artery (LPA) stenosis (invasive gradient 20 mmHg). Right ventricular volumes and function were preserved. The Ea and Ees were increased but RV-PA coupling was relatively maintained. Of interest, RV end-diastolic pressure and RV diastolic stiffness were increased. After LPA plasty, RV function was preserved during long-term follow-up. Patient 2 was symptomatic despite mildly elevated RV pressures and a supravalvular RV-PA conduit stenosis (invasive gradient 30 mmHg). The RV showed severe RV dilatation and dysfunction. The Ea was increased but Ees was decreased leading to RV-PA uncoupling. Despite balloon angioplasty, RV function was unchanged during long-term follow-up.
UNASSIGNED: Development of RV dysfunction might be insufficiently predicted by RVOTO severity in patients with repaired ToF. Assessment of RV remodelling and function in relationship to its afterload might help to optimize risk stratification.

BACKGROUND: We discuss a rare case of an adult patient with different pathologies involving the aortic and pulmonary valves in need of surgery.
METHODS: The patient had a history of congenital PV stenosis and surgical valvuloplasty. Almost 50 years later the patient underwent a complex second heart surgery due to infective endocarditis of the aortic valve and high-grade restenosis of the pulmonary valve. Replacement of the aortic and pulmonary valve, as well as reconstruction of the RVOT and closure of a persistent foramen ovale, followed. Postoperative course was uneventful and the patient was discharged home a week after surgery.
CONCLUSIONS: Simultaneous surgery of pulmonary and aortic valves due to different pathologies is rare but can be performed successfully even in advanced age and can improve quality of life.

A 9-month-old infant developed pulmonary stenosis (PS) after an arterial switch operation for transposition of the great arteries, accompanied by a Shaher Type 4 coronary anatomy. As the right coronary artery (RCA) ran across the anterior side of the right ventricle (RV), atrioventricular (AV) groove patch plasty was performed to relieve PS. The distance between the RCA and tricuspid valve was confirmed by preoperative-computed tomography. The AV groove was carefully incised, ensuring the position of the tricuspid valve, and maintaining a distance of 3 mm from the tricuspid annulus to avoid approaching the RCA. While suturing the monocuspid valve patch, only the endocardial side of the RV was sutured, and RCA injury was prevented. Thus, especially in patients < 1 year of age, careful incision of the AV groove and suturing only the endocardial side is important to avoid injuring the RCA in AV groove patch plasty.

Many traits and phenotypic characteristics present in the human body such as height, skin, pigmentation, hair, and eye color are inherited through many alleles present in different loci. This is known as polygenic inheritance. Congenital rubella syndrome (CRS) is a rare disease characterized by congenital deformities such as chorioretinitis and cataracts. This disease is endemic in India, and it is mainly caused by the rubella virus. We report a case of a 4.5-year-old female child who presented with breathlessness and radiolucent bone disease, for which she was already undergoing surgical interventions. The cell culture line was positive for rubella. The patient was treated with oxygen therapy via continuous positive airway pressure (CPAP) and had a moderate biventricular function.

Hedinger Syndrome or carcinoid heart disease is a rare cardiac complication of neuroendocrine tumors (NET) affecting the tricuspid and pulmonary valves. Following is a case description of a patient undergoing treatment for a neuroendocrine tumor with liver metastasis, referred with symptomatic tricuspid valve regurgitation and pulmonary valve stenosis for surgical valve replacement. Planned surgical valve replacement was successfully performed before the onset of severe right ventricular failure or pulmonary hypertension in this case of carcinoid heart disease. An interdisciplinary approach and regular follow up is recommended in such cases.

Quadricuspid pulmonary valve (QPV) is a rare congenital anomaly. Simple QPV had been mainly diagnosed at the time of autopsy before 2000, and the frequency rates of QPV are approximately 0.02%-0.41%. QPV was initially diagnosed using transthoracic echocardiography (TTE) after 2000 and with contrast computed tomography (CT) or cardiac magnetic resonance imaging (CMR) after 2009. Obtaining the cross-sectional view of the pulmonary valve using TTE is difficult. We aimed to review the papers regarding the incidence, embryology, diagnosis, associated congenital heart anomalies, and prognosis in patients with QPV, and furthermore to compare with those in patients with quadricuspid aortic valve (QAV).
We diagnosed QPV with mild stenosis in a 12-month-old infant. With a slight angulation of the transducer superiorly from the left high parasternal short-axis view, a short-axis view of QPV was obtained.
In QPV cases diagnosed at autopsy, Hurwitz\'s type-b with three equal cusps and one smaller cusp is dominant, whereas Hurwitz\'s type-a with four equal cusps is dominant in clinically diagnosed cases. Congenital heart anomaly and valvular stenosis are more frequent in patients with QPV than in patients with QAV. Coronary artery anomalies and infectious endocarditis are more frequent in patients with QAV than in patients with QPV. The incidence of PR is more common in type-a QPV than in type-b QPV. There is no difference between type-a QAV and type-b QAV with respect to the incidence of aortic regurgitation (AR). It is assumed that QPV is a risk factor for a Ross operation. However, QPVs have been used as autografts in certain patients.
Between QPV and QAV, various differences were found in frequency rates, diagnostic methods, valve morphology, valve function, associated congenital heart diseases, and frequencies of infectious endocarditis.

BACKGROUND: Patients with congenitally corrected transposition of great arteries (ccTGA) not infrequently seek medical attention for the first time late in life. Optimal management of natural history ccTGA is debated and must be tailored.
METHODS: A 38-year-old male patient was referred to our centre because of severe cyanosis and worsening dyspnoea. Investigations disclosed situs solitus, mesocardia, double discordance, large ventricular septal defect (VSD), severe pulmonary stenosis, and no significant atrio-ventricular valves regurgitation. The patient underwent physiologic repair: VSD closure, placement of a left ventricle to pulmonary artery conduit, and epicardial atrio-biventricular pacemaker implantation. The conduit was intentionally undersized to promote tricuspid valve continence. Post-operative course was uneventful, transthoracic echocardiography showed good biventricular function without significant tricuspid regurgitation. At 1 month after discharge, the patient is in New York Heart Association Class II.
CONCLUSIONS: Management of late presenter patients with ccTGA depends on the associated lesion and estimation of surgical risk. In selected patients markedly symptomatic physiologic repair is a rationale option, providing a normal saturation and biventricular circulation with a significantly lower surgical risk as compared with an anatomic repair.

Double-outlet right ventricle (DORV) is a rare cardiac condition in which both the aorta and pulmonary artery arise from the right ventricle, resulting in parallel systemic and pulmonary circulations. Usually, DORV is present with ventricular septal defect; however, the location of the ventricular septal defect and presence of pulmonary stenosis (PS) result in various physiological features and subtypes of DORV. Because DORV without PS causes congestive cardiac failure and DORV with PS results in cyanotic heart disease, anesthesia management varies widely according to the resultant physiological characteristics. Reports of anesthesia management in a parturient with DORV undergoing cesarean delivery is scarce because of the low incidence of DORV and the discouragement of these patients to conceive. Only 8 known previous such cases are reported, and almost all these patients were administered regional anesthesia. Here we describe a parturient with DORV, to whom general anesthesia was administered because of incidental antiphospholipid syndrome with low platelets. To the best of our knowledge, this scenario has not been described previously.