Treatment of advanced heart failure in adults with congenital heart disease: a narrative review and clinical cases.

Background and Objective: The number of adults with congenital heart disease (ACHD) is increasing worldwide. Almost all congenital cardiac lesions can be successfully treated due to the progress in neonatal surgery and pediatric cardiology with a high likelihood of surviving until adulthood. However, ACHD frequently develop sequelae related to the initial cardiac anomaly. Heart failure (HF) is one of the most common complications associated with a high morbidity and mortality. Methods: The authors did search the PubMed database regarding relevant content covering publications up to March 2022. Relevant manuscripts were classified according to the impact factor of the journal, being a guideline manuscript, a position paper by a society or a comprehensive review of the current literature. Key Content and Findings: Optimal HF treatment remains an unmet need in ACHD. In particular, advanced HF therapy with cardiac resynchronization therapy, ventricular assist devices or organ transplantation is still very different and more specific in ACHD compared to non-ACHD. This review aims to compile international views and evidence from the literatures on the treatment of advanced HF in ACHD. Current challenges, but also the success of different treatment strategies in ACHD are illustrated by clinical cases. Conclusions: The main finding of the review is that data is still scarce regarding ACHD with advanced HF and international efforts to collect data regarding these patients needed to improve the current standard of care.

Cardiac Inflammatory Myofibroblastic Tumor Causing Pulmonary Artery Obstruction: A Rare Case Report.

Inflammatory myofibroblastic tumor (IMT) is a rare soft tissue tumor of the heart. In the literature, cardiac IMT is often described as an endocardial-based cavitary mass originating from the right side of the heart in infants and adolescents. In this article, we present a 5-year-old boy with a rare cardiac IMT who had no complaints and was diagnosed with murmur during his routine examination. Transthoracic echocardiography showed a homogeneous polypoid mass originating from the pulmonary valve, extending into the main pulmonary artery during systole and causing obstruction of the pulmonary artery and right ventricular outflow tract. Surgical resection of the tumor was performed successfully. There was no tumor recurrence in the control echocardiography at the postoperative first month.

Heart failure in cardiomyopathies: a position paper from the Heart Failure Association of the European Society of Cardiology.

Cardiomyopathies are a heterogeneous group of heart muscle diseases and an important cause of heart failure (HF). Current knowledge on incidence, pathophysiology and natural history of HF in cardiomyopathies is limited, and distinct features of their therapeutic responses have not been systematically addressed. Therefore, this position paper focuses on epidemiology, pathophysiology, natural history and latest developments in treatment of HF in patients with dilated (DCM), hypertrophic (HCM) and restrictive (RCM) cardiomyopathies. In DCM, HF with reduced ejection fraction (HFrEF) has high incidence and prevalence and represents the most frequent cause of death, despite improvements in treatment. In addition, advanced HF in DCM is one of the leading indications for heart transplantation. In HCM, HF with preserved ejection (HFpEF) affects most patients with obstructive, and ∼10% of patients with non-obstructive HCM. A timely treatment is important, since development of advanced HF, although rare in HCM, portends a poor prognosis. In RCM, HFpEF is common, while HFrEF occurs later and more frequently in amyloidosis or iron overload/haemochromatosis. Irrespective of RCM aetiology, HF is a harbinger of a poor outcome. Recent advances in our understanding of the mechanisms underlying the development of HF in cardiomyopathies have significant implications for therapeutic decision-making. In addition, new aetiology-specific treatment options (e.g. enzyme replacement therapy, transthyretin stabilizers, immunoadsorption, immunotherapy, etc.) have shown a potential to improve outcomes. Still, causative therapies of many cardiomyopathies are lacking, highlighting the need for the development of effective strategies to prevent and treat HF in cardiomyopathies.

Single centre experience of combat-related vascular injury in victims of Syrian conflict: Retrospective evaluation of risk factors associated with amputation.

OBJECTIVE: To elucidate the risk factors associated with amputation in cases with combat-related vascular injury (CRVI). MATERIAL AND METHODS: This retrospective study included 90 cases with CRVI treated between May 2011 and July 2013. The patients were divided into group I (n=69), in which the limb was salvaged and group II (n=21), in which the patients received amputation. RESULTS: The overall and the secondary amputation rates were 23% and 18%, respectively. There were no amputations with the MESS of nine or less, increasing proportions of amputations at 10 and 11, with a level of 12 leading to 100% amputation rate. The mortality rate was 2%. Among the 52 (58%) cases with the mangled extremity severity score (MESS) ≥7, the limb salvage rate was 60%. The patients in group II were more likely to have a combined artery and vein injury (p=0.042). They were also more likely to be injured as a result of an explosion (p=0.004). Along with the MESS (p<0.001), the duration of ischemia (DoI) (p<0.001) were higher in group II. The rate of bony fracture (p<0.001) and wound infection (p=0.011) were higher in group II. For the overall amputation, the odds ratio of the bony fracture (OR: 61.39, p=0.011), nerve injury (OR: 136.23, p=0.004), DoI (OR: 2.03, p=0.003), vascular ligation (OR: 8.65, p=0.040) and explosive device injury (OR: 10.8, p=0.041) were significant. Although the DoI (p<0.001) and the MESS (p=0.004) were higher in whom a temporary vascular shunt (TVS) was applied, the utilisation of a TVS did not influence the amputation rate (p=1.0). CONCLUSIONS: The DoI and the variables indicating the extent of tissue disruption were the major determinants of amputation. While statistically non-significant, the benefit of the application of a TVS is non-negligible. MESS is a valid scoring system but should not be the sole foundation for deciding on amputation. Extremities which were doomed to amputation with the MESS>7 seem to benefit from revascularisation with initiation of reperfusion at once. The validity of MESS merits further investigation with regard to the determination of a new cut-off value under ever developing medical management strategies.

Simultaneous occurrence of a large asymptomatic prolapsing left atrial myxoma with a cutaneous squamous cell carcinoma.

Synchronous myxoma of the heart and other malignancies are extremely rare. We report a case of a 64-year-old man who had a large left atrial myxoma that obstructed the mitral valve, as well as an unrelated, coexistent cutaneous squamous cell carcinoma in the sacral area. During the preoperative evaluation for non-cardiac surgery, the tumor was diagnosed coincidentally by echocardiographic examination. Echocardiography findings were consistent with a large left atrial myxoma originating from the posterior wall and prolapsing into the left ventricular cavity through the mitral valve, causing mitral stenosis. The mass was successfully completely excised. Histologic examination of the mass confirmed the diagnosis of cardiac myxoma. We report a casual echocardiographic finding of a left atrial myxoma that obstructed the mitral valve outflow tract, and an unrelated, synchronous cutaneous squamous cell carcinoma in the sacral area.

A mandatory modification in extracorporeal biventricular assist device (BIVAD) implantation: intercostal tunnel application: a case report.

In this case, our patient was a heart transplant candidate connected to a respiratory system. An extracorporeal biventricular assist device (BIVAD) was the only option in order to bridge to transplantation. In routine procedures, it is recommended that Berlin Heart Excor cannulas be removed through the subfascial subcostal tunnel. As the severely dilated right ventricle compressed the apex of the left ventricle, which was also dilated to the mid-back zone of the left hemithorax, the whole length of the Extracorporeal BIVAD apical cannula had to remain within the thorax; however, the cannula was removed from the body by creating a tunnel at the 7th intercostal space. In the long-term follow-up, this compulsory modification has proven to be safe and effective.

Ligation of patent ductus arteriosus in low birth weight premature infants: timing for intervention and effectiveness of bed-side surgery.

BACKGROUND: Patent ductus arteriosus is a common congenital cardiac condition. Its importance is mostly underestimated and accepted as an "easy" heart disease. Physiological consequences of pulmonary overflow may cause severe mortality in premature neonates. Accurate timing of surgical intervention is essential to decrease the mortality in very low birth weight premature infants. On-site surgery in the intensive care units (ICUs) results excellent surgical quality without jeopardizing the safety of the patients. METHODS: We have summarized the clinical and operative data of 26 premature neonates (<37 weeks of gestational age), which were operated for the diagnosis of PDA in the ICUs of Dokuz Eylül University. Thirteen low birth weight infants (<1000 gr) have been compared with remaining 13 neonates (>1000 gr). RESULTS: There was no surgical mortality in both groups. Co-existing problems were observed in both groups, which did not affect surgical mortality and morbidity. CONCLUSIONS: Surgery in the ICU is a safe method for premature neonates with physiologically significant PDA. This technique should be the method of choice in experienced centers.

Intrapericardial teratoma in a newborn: a case report.

We report a successfully surgically intervened case of intrapericardial teratoma, which was diagnosed prenatally. Intrapericardial teratomas are rare cases, and surgical management of those tumors are challenging in this age group. The compression effect of the mass led to misdiagnosis of the anomaly as a transposition of the great vessels. We conclude that intrauterine echocardiography in experienced hands is an essential tool for the follow-up of these patients to detect the pericardial effusion and compression of the cardiac structures, which may cause tamponade and heart failure.

A congenital arteriovenous malformation originating from the aorta locating in the posterior mediastinum.

Vascular malformations located in the posterior mediastinum are extremely rare. Most of them are found coincidentally during routine examinations. Only a small percentage of these posterior mediastinal arteriovenous malformation cases may cause symptoms such as dyspnea due to compression of surrounding tissues. Radiologic imaging can be insufficient in some cases for differential diagnosis. Because of their vascular nature, diagnostic needle biopsy may have a high risk of bleeding. Open surgical resection is a safe treatment choice under many circumstances, and it helps the diagnosis as well. In this paper, a case of a 31-year-old male is presented with an incidentally diagnosed arteriovenous malformation, originating from the descending aorta and located in the posterior mediastinum.

Vascular rings: presentation, imaging strategies, treatment, and outcome.

This study aimed to evaluate the presenting symptoms, the effectiveness of imaging methods, and the surgical treatment of vascular rings. Data for 44 patients (32 enrolled prospectively, 12 reviewed retrospectively) over a 10-year period in a tertiary referral center were analyzed. These patients comprised 25 patients with a left aortic arch and an aberrant right subclavian artery, 13 patients with a right aortic arch and a left subclavian artery originating from Kommerell's diverticulum, 1 patient with a right aortic arch and an aberrant left subclavian artery, 3 patients with a double aortic arch, and 2 patients with a pulmonary sling. Respiratory symptoms were found in 25 patients and dysphagia in 6 patients. Atypical symptoms such as reflex apnea, cyanosis, syncope episodes, and exercise-induced wheezing were noted in five patients. Associated congenital heart defects were detected in 41% of the patients. The diagnostic yield was 95.23% for barium esophagography, 54.54% for echocardiography, and 66.66% for computed tomography. The anatomy could be correctly identified by magnetic resonance imaging (MRI) in 97.43% and by angiography in 90.5% of the patients. Of the 30 patients who underwent surgery, 80% were completely relieved of symptoms during a mean follow-up period of 25 ± 33.5 months. Vascular rings should not be overlooked in infants with atypical symptoms. The authors' diagnostic procedure of choice is MRI because it is superior to angiography for delineating the relationship between abnormal vascular structures, trachea, and esophagus.

Physical therapy in the intensive care unit in a patient with biventricular assist device.

PURPOSE: To determine the safety and feasibility of early physical therapy in the intensive care unit in a patient with biventricular assist device. METHODS: Physical therapy started on the first postoperative day and continued till discharge including airway clearance, lower/upper extremity exercises, and mobilization. Heart rate (HR), respiratory rate (RR), systolic/diastolic/mean arterial pressures, peripheral oxygen saturation, and double product were recorded before treatment, after treatment, and 5 minutes after treatment. RESULTS: In total, 15 sessions of physical therapy were implemented for a 41-year-old male patient during 21 days following implantation of a biventricular assist device. Normal physiological responses were seen in response to treatment. Heart rate increased significantly after treatment in comparison to pretreatment values (p = 0.02) and decreased significantly after 5 minutes (p = 0.03) and approached pretreatment values. Respiratory rate increased nonsignificantly after treatment and decreased significantly after 5 minutes (p = 0.001) and approached pretreatment values. CONCLUSION: Physical therapy in the intensive care unit in a patient with biventricular assist device resulted in significant increases within HR and RR in physiological limits. Ongoing monitoring of vital signs is recommended in order to observe physiological responses to early physical therapeutic interventions in the intensive care unit.

Postcardiotomy extracorporeal life support.

The need for postcardiotomy mechanical support is uncommon and likely to decline. A mixture of options is necessary to meet the diverse indications for cardiac support in a comprehensive heart failure program. Between January 1997 and December 2000, 29 adult, neonate, and infant cardiac surgical patients were supported on an extracorporeal life support system. Indications for cardiac assist included post-cardiotomy low cardiac output syndrome, and hyperacute rejection after cardiac transplantation. Data for analysis were collected prospectively. Survival on the life support system was 20/29 (69%) and 12 patients (41%) survived to discharge. The mean time to starting extracorporeal life support was longer in survivors than non-survivors. The extracorporeal life support system provides effective cardiopulmonary and end-organ support.

Surgical resection for successful treatment of invasive pulmonary aspergillosis: report of 3 cases.

We present 3 patients, 2 with recent heart transplants, complicated with invasive pulmonary aspergillosis (IPA), treated successfully with surgical resection. These patients demonstrate the role of surgery in management of IPA, and 2 heart transplant patients are of particular interest as surgical treatment of IPA after solid organ transplantation is seldom reported.

Pleural flap for treating perigraft leak after a modified Blalock-Taussig shunt.

Plasa oozing through the graft after a modified Blalock-Taussig shunt is a troublesome complication. We encountered a massive leak following a modified Blalock-Taussig shunt in a 2 1/2 year-old-girl which required reexploration. The leak was treated by wrapping the polytetrafluoroethylene shunt with the parietal pleura flap harvested from the adjacent chest wall. The patient had an uneventful recovery. Covering of the polytetrafluoroethylene shunt with parietal pleura appears to stop plasma leak through the graft following a modified Blalock-Taussig shunt.