The Road to Heart Transplant in a Patient With Cardiomyopathy, Shone Complex, and Severe Pulmonary Hypertension.

Our case report details the journey of a 16-year-old male patient with Shone complex and advanced heart failure. We highlight the pivotal role of the HeartWare Ventricular Assist Device (Medtronic) implantation in mitigating severe pulmonary hypertension, thereby facilitating his eligibility for a heart transplant. We discuss the subsequent management of post-transplant pulmonary hypertension and right ventricular dysfunction using targeted pulmonary vasodilators and inotropic support, underscoring the intricacies of postoperative care in pediatric heart transplant patients. This case emphasizes our observation of the critical role that left ventricular assist devices play in redefining transplant candidacy and the necessity for complex, ongoing management in pediatric heart transplant scenarios.

Non-syndromic Parachute Mitral Valve "When the Valve Dives in": Case Report and Review of the Literature.

A parachute mitral valve (PMV) is a congenital mitral valve anomaly diagnosed in infancy, and it can also be discovered in adults during echocardiography. Surgical management is common in infants to prevent complications from left-heart obstructions. In adults, PMV may be found independently or with other cardiac defects. Prophylactic antibiotics are recommended for certain congenital heart anomalies before dental procedures. A study suggests reconsidering guidelines to include anomalies like bicuspid aortic valve and MVP for antibiotic prophylaxis. PMV, with transvalvular blood flow turbulence, may increase the risk of infective endocarditis, as seen in a reported case with a parachute-like mitral valve. Here, we present the case of a 62-year-old female incidentally found to have a PMV during an echocardiogram.

An Undiagnosed Shone Complex in a 52-Year-Old Female: A Case Report.

Shone complex (SC) is a rare congenital heart disease characterized by four obstructive anomalies, including parachute mitral valve (PMV), left atrial supra-valvular ring, subaortic stenosis, and coarctation of the aorta. Typically, SC manifests early in life. However, we encountered a 52-year-old female with a history of hypertension diagnosed at 26 years and left-sided weakness poststroke. She presented with worsening dyspnea and palpitations, prompting a thorough investigation. Echocardiography revealed a heavily calcified bicuspid aortic valve with severe aortic stenosis and parachute mitral valve with severe mitral stenosis and preserved ejection fraction, raising suspicions regarding the presence of SC. Cardiac catheterization, aortic-angiography, and noncontrast chest computed tomography (CT) revealed abrupt occlusion of the postductal aorta, giving a picture of aortic coarctation with well-established collateral vessels including prominent right and left internal mammary arteries. So, she was diagnosed with an incomplete SC at the age of 52. Shone complex is a rare congenital heart disease that typically presents in early childhood, but late presentations due to misdiagnosis or incomplete work up are possible. This case emphasizes the rarity of late presentations of SC and highlights the importance of early diagnosis and intervention to improve outcomes. An incomplete SC should be considered in adult patients presenting with left-sided obstructive lesions.

Perioperative and Anesthetic Considerations in Shone's Complex.

Shone's complex is a congenital cardiac disease consisting of the following four lesions: parachute mitral valve, supravalvar mitral ring, subaortic stenosis, and aortic coarctation. Though not all components are required for a diagnosis, the end result is both left ventricular inflow and outflow obstruction, which typically present in patients as congestive heart failure. The complex pathology requires careful management and surgical decision-making to ensure an optimal outcome. This review will focus on the anatomy, physiology, and perioperative anesthetic management of patients with Shone's complex.

[Rare case of incomplete Shone syndrome in adult man with marfanoid morphotype]. / Un cas rare de complexe de Shone incomplet chez un homme de 35 ans avec un morphotype marfanoïde et revue de la littérature.

Shone syndrome is a rare and complex congenital heart disease. It is characterized in its complete form, by a constellation of the four potentially obstructive lesions of the left heart side. Incomplete Shone's complex includes 2 or 3 of the characteristic lesions which, include a supravalvular mitral membrane, valvular mitral stenosis (MS) by a parachute mitral valve (PMV), subaortic stenosis and aortic coarctation (Coa). We describe here, the case of a 35-year-old man, hospitalized for chronic dyspnea and in whom, the diagnosis of incomplete Shone's complex is retained by echocardiographic analysis. Observed lesions include a parachute mitral valve responsible for mild regurgitation and moderate mitral stenosis, bicuspid aortic valve, and pseudo interruption of the aorta with dilatation of the ascending aorta and a patent ductus arteriosus. This case is the 2nd on 3942 patients in Drighil congenital heart disease registry.

Single coronary artery and coronary artery-pulmonary artery fistula in a variation of the Shone complex.

We aim to share the diagnostic methods and relevant findings of a rare case involving a 1-year-old girl with a variation of Shone syndrome, presenting with the coexistence of a single coronary artery and a fistula between the coronary artery and the right pulmonary artery. The patient had previously undergone aortic arch reconstruction surgery and during the preoperative evaluation, cardiac computed tomography angiography (CCTA) revealed the additional presence of a single coronary artery originating from the right, which fistulized into the right pulmonary artery distally. This finding was further confirmed by conventional angiography. This case report highlights the importance of CCTA in identifying and directing the treatment of additional anomalies that may accompany rare congenital abnormalities.

Prenatal prediction of Shone's complex. The role of the degree of ventricular disproportion and speckle-tracking analysis.

OBJECTIVES: Shone's complex (SC) is characterized by sequential obstructions of left ventricular (LV) inflow and outflow. It can be associated with poor long-term prognosis when compared to Simple-Aortic Coarctation (S-CoA). We aimed to assess whether the degree of ventricular disproportion and 2D-speckle-tracking echocardiography (2D-STE) could improve the accuracy of prenatal prediction of SC. METHODS: 75 consecutive fetuses were retrospectively enrolled from January 2010 to June 2021. Fetuses were divided into 4 groups (Group 1: SC; Group 2: S-CoA; Group 3: False Positive-Coarctation of the Aorta [FP-CoA]; group 4: controls). Comparisons for echocardiographic measures and myocardial deformation indices were performed. A receiver operating characteristic (ROC) analysis was performed on the MV/TV (mitral valve/tricuspid valve ratio) and LV GLS (global longitudinal strain) values to identify cut-offs to separate group 1 and 2 fetuses. RESULTS: SC fetuses showed a significant reduction in MV/TV when compared to S-CoA and FP-CoA fetuses (p<0.001). LV GLS in SC fetuses was significantly reduced compared to S-CoA fetuses (-13.3 ± 2.1% vs. -17.0 ± 2.2%, p=0.001). A cut-off value of 0.59 for MV/TV and -15.35% for LV GLS yielded a sensitivity of 76 and 82% and a specificity of 71 and 83% respectively in separating SC vs. S-CoA fetuses. CONCLUSIONS: SC fetuses showed a more severe degree of ventricular disproportion and a lower LV GLS compared to S-CoA, FP-CoA and control fetuses. MV/TV and GLS are both predictors of SC. These findings may improve the quality of prenatal parental counselling.

Shone's complex and aortic dissection: case report and review of a rare, underdiagnosed congenital heart disease.

BACKGROUND: Shone's complex is a rare congenital heart disease consisting of a variety of left ventricular inflow and outflow tract lesions. Patients typically present in childhood requiring early surgical intervention; however, with improved surgical techniques, these patients are surviving later into adulthood. This increased survival comes with a new set of medical complications that providers need to be aware of. CASE PRESENTATION: A 27 year old man with a complex cardiac history including an incomplete Shone's complex and persistent symptomatic atrial flutter presented with sharp chest pain radiating to his back. He was found to have type A aortic dissection on imaging in the setting of severe patient-prosthesis mismatch. He had multiple valvular surgeries in childhood. The patient was being followed-up as an outpatient for an enlarging chronic aortic aneurysm and was non-compliant with his medications. He was taken emergently to the operating room for a skirted Bentall procedure, aortic valve replacement, and right sided MAZE. CONCLUSIONS: Shone's complex is a rare congenital heart disease associated with significant morbidities including atrial flutter, patient-prosthesis mismatch, and aortic dissection. As patients continue to live longer into adulthood with this disease, it is important to raise awareness of this rare syndrome for providers and highlight its potential complications. Further research is needed to determine appropriate guidelines for when to intervene on aortopathy-associated CHD.

Atypical Shone's Complex Diagnosed at 70 Years Old: Presenting with Double-orifice Mitral Valve, Bicuspid Aortic Valve, and Aortic Coarctation.

Atypical Shone's complex is a rare congenital anomaly involving a left-sided obstructive lesion of two or three cardiovascular levels. A 70-year-old man with dyspnea on exertion was diagnosed with severe aortic stenosis (AS) with a bicuspid valve, complicated by severe aortic coarctation (CoA) and a double-orifice mitral valve. He underwent surgery for AS and CoA in one session. It is important to search for complicated malformations, even in cases of bicuspid aortic valve found in old age.

Strategies to mitigate inflammation in management of complex congenital heart disease complicated by "multisystem inflammatory syndrome in children".

A 6-month-old boy, a case of Shone's complex, presented in decompensated state was found to have severe mitral stenosis along with multisystem inflammatory syndrome in children (MISC) warranting urgent surgical intervention. Various modalities including cytokine-adsorbing hemofilter were used to target inflammation. Postoperatively, the child recovered from low cardiac output accompanied by decrease in the levels of inflammatory markers, inopressors, and ventilatory requirements. Open heart surgery in itself is a proinflammatory process and is best avoided during the active inflammatory phase of MISC. In the rare and unavoidable circumstance exemplified by this index case, multipronged strategy targeting inflammation as described can be successfully implemented.

Evaluation of midterm outcomes after Shone's complex surgery: Analysis of reoperation and mortality risk factors.

OBJECTIVE: The midterm results of patients who underwent biventricular repair surgery for Shone's complex were examined, and mortality and reoperation risk factors were evaluated. METHODS: This retrospective study included 34 patients with Shone's complex who underwent mitral valve (MV) surgery between 2005 and 2020. RESULTS: A total of 19 patients (56%) had coarctation, 10 (29%) patients had subaortic stenosis, 9 (26.5%) patients had a hypoplastic aortic arch (AA), and 9 (26.5%) patients had aortic valve (AV) stenosis. Twenty-four (70.6%) patients had bileaflet AV. Associated left-sided in-flow stenotic lesions included parachute MV in 19 (56%) patients and supramitral ring in 18 (53%) patients. The estimated freedom from reoperation rate on the 6th month, 1 year and 2 years after surgery was 84.4%, 79.5%, and 71.5%, respectively. The overall mortality rate was 20.6% (seven patients) with a median follow-up of 10 months (0-41). The estimated survival rate on the 6th month, 1 year, and 3 years after surgery was 83.8%, 79.4%, and 79.4 respectively. Bicuspid aortic valve (p = .017) (HR (95% CI) = 0.130 (0.025-0.695) and hammock mitral valve (p = .038) (HR (95% CI) = 11,008 (1,146->100) were associated with mortality. CONCLUSION: The presence of a bicuspid aortic valve hammock mitral valve might have an effect on negative effect on the outcome.

The Appearance of an Underrecognized Congenital Heart Disease in a Patient With COVID-19 Pneumonia.

A 60-year-old woman presented to the emergency department with worsening shortness of breath and non-productive cough for 1 week, which was preceding a recent COVID-19 infection. At the time the patient thought this was part of the constellation of symptoms of COVID-19, so she stayed home until her symptoms worsened to the point of needing hospitalization. The patient was found to have a rare and complex congenital heart disease that led her to develop acute heart failure precipitated by COVID-19 pneumonia. Medical management and surgical repair were essential in this patient given the late presentation.

An unusual case of Shone's complex in an adult depicted on computed tomography angiography.

We present a case of a 22-year-old male with dyspnea on exertion where computed tomography revealed complete Shone's complex. This case highlights the complementary role of computed tomography in the anatomical evaluation of patients with complex heart diseases.

Outcomes of biventricular repair for shone's complex.

BACKGROUND: Shone's complex is a rare lesion affecting the mitral valve (MV) and left ventricular outflow tract (LVOT). The objective of this study is to report the outcomes after Shone's complex repair, the growth of mitral and aortic valve and LVOT, and long-term survival. METHODS: This retrospective study included all patients diagnosed with Shone's complex, who underwent biventricular repair. Data including patients' characteristics, type of the MV lesion and the associated lesions were collected. Patients were followed up regularly with echocardiography, and the changes in mitral and aortic valve z-score and LVOT z-score were recorded. RESULTS: Thirty-seven patients were included in the study, the median age was 3.4 months, and 11 patients (30.6%) had pulmonary hypertension. The main procedure performed during the first surgical intervention was coarctation repair in 26 patients (70%). Twelve patients had MV repair, and five had MV replacement. Operative mortality occurred in 1 patient (2.7%), median follow up was 52 (25-75th percentile: 22-84) months. Survival at 1, 5, and 10 years was 94.4%, 90%, and 76.9%, respectively. Reoperation was required in 13 patients, mainly for LVOT repair (n = 8). Reoperation was significantly associated with associated aortic valve lesion (p = .044). The growth of the MV z-score was 0.35 per year; p < .001, aortic valve z-score 0.086 per year; p = 0.422, and the LVOT z-score was 0.53 per year; p = .01. CONCLUSION: Biventricular repair of Shone's complex has good outcomes. Reoperation is frequently encountered, especially with low aortic valve z-score. The MV and LVOT have significant growth following Shone's complex repair.

Management of Severe Coarctation of the Aorta During Pregnancy.

An 18-year-old primigravida woman underwent emergent percutaneous balloon stent repair of a severe coarctation of the aorta, allowing her to undergo an uneventful remaining pregnancy and the delivery of a healthy baby. Her case also demonstrates the association between maternal coarctation and fetal Shone complex. (Level of Difficulty: Beginner.).

Role of perioperative echocardiography in repair of incomplete shone complex: A case series.

Multilevel obstruction of left-sided heart structures was originally characterized by Shone et al. The formulation of an appropriate operative strategy remains challenging and needs to be individualized for this complex subset of patients. Intraoperative transesophageal echocardiography (TEE) not only helps in delineating spatial anatomy but also reveals associated anomalies that help in decision-making regarding operative strategies for these patients. Here, we discuss five such cases of Shone's anomaly presenting at varied age group with different associated anomaly in which intraoperative TEE played a pivotal role in the management.

Age-related changes in diastolic function in children: Echocardiographic association with vortex formation time.

BACKGROUND: This study aims to understand the age-related changes in vortex formation time (VFT) index in children, and thus, describe the ranges of VFT in different pediatric age groups with the ultimate goal of assessment of diastolic function. METHODS AND RESULTS: Transthoracic echocardiograms in healthy (n = 84) subjects from birth to 20 years were analyzed to compute VFT and diastolic performance. LV apical and short-axis views were used. Three separate measurements were performed, and the mean was used to derive VFT and other indices. Statistical comparisons were made amongst the groups, stratified by age. RESULTS: Vortex formation times in neonates (median 1.79, interquartile range 1.31-1.92) and infants (1.38, 1.07-1.72) were found to be significantly lower (P < .05) than the older age groups (1-5 years 2.47, 1.87-2.94, 5-10 years 2.18, 1.89-2.53, 10-20 years 2.34, 1.84-2.96). The changes in VFT correlate to the changes in diastolic function in children. CONCLUSION: Our results show that unlike adults, VFT changes along with the growth-related myocardial adaptations in children, and its range may be used to evaluate diastolic function. The present study is the first to test the significance of the trans-mitral VFT in children by comparing different age groups of healthy subjects.

Short-term results in infants with multiple left heart obstructive lesions.

OBJECTIVE: Deciding on a surgical pathway for neonates with ≥2 left heart obstructive lesions is complex. Predictors of the successful biventricular (2V) repair in these patients are poorly defined. The goal of our study was to identify patients who underwent the 2V repair and assess anatomic and echocardiographic predictors of success. DESIGN: Infants born between July 2015 and August 2017 with ≥2 left heart obstructive lesions with no prior interventions were identified (n = 19). Patients with aortic or mitral valve (MV) atresia and critical aortic stenosis were excluded. Initial echocardiograms were reviewed for aortic, MV, tricuspid valve annulus size, and left (LV) and right (RV) ventricle diastolic longitudinal dimensions. The valve morphology and presence of a ventricular septal defect (VSD) and coarctation were assessed. Clinical outcomes included successful 2V repair, complications, and repeat interventions or surgeries. Failed 2V repair was defined as a takedown to single ventricle (1V) physiology, cardiac transplantation, or death. RESULTS: For 2V repair, 14/19 patients were selected and for 1V, 5/19 patients were selected. Initial surgical procedures of the 2V group were simple coarctation repair (5), complex coarctation/arch reconstruction +/- septal defect closure (6), hybrid stage 1 (2), and none (1). Three of the 2V patients required reintervention in the first 90 days. The LV to RV diastolic longitudinal ratio >0.75 and mitral/tricuspid ratio of <0.8 were observed in 13/14 of the 2V patients. The LV:RV ratio and the aortic valve z score were significantly larger in the 2V group compared to the 1V group. All patients in the 1V group had a nonapex forming LV. There was no mortality with follow-up to three years of age. CONCLUSIONS: This study showed excellent short-term and midterm surgical results in the 2V population. The LV:RV diastolic longitudinal ratio may be a useful tool in the risk stratification of a successful 2V repair even in cases with a small MV.

Incomplete Form of Shone Complex in an Adult Congenital Heart Disease Patient.

Shone complex is a rare congenital disorder that involves 4 obstructive lesions of the left heart, as follows: parachute mitral valve, supravalvular mitral ring, subaortic stenosis, and coarctation of the aorta. Incomplete forms with 2 or 3 of these lesions in adult patients have been rarely reported in the literature, meaning that insufficient general data exist concerning the surgical strategy and clinical follow-up. Herein, we report the case of a 31-year-old woman with a diagnosis of incomplete form of Shone complex with parachute mitral valve and coarctation of the aorta who underwent successful single-stage surgical repair.