The association between myocardial ischemia and myocardial dysfunction in adult patients with systemic right ventricle - A single centre multimodality study.

BACKGROUND: The exact interaction of factors leading to myocardial dysfunction and fibrosis of the systemic right ventricle (SRV) is not completely understood. Myocardial ischemia and injury associated with a supply-demand mismatch of the pressure overloaded SRV are thought to play an important role, however studies confirming this are lacking. METHODS: Adult SRV patients were included in this single centre cohort study. All patients underwent a comprehensive diagnostic and imaging workup. A two-day stress-rest SPECT was performed to assess myocardial perfusion. SRV ischemia was defined as decreased segmental tracer uptake during exercise with significant improvement at rest. Contrast enhanced cardiac magnetic resonance imaging (CMR) was also performed in a subgroup of patients without contraindication, to assess focal myocardial fibrosis. Differences between patients with and without SRV ischemia were assessed. RESULTS: Twenty-three SRV patients (15 with transposition of the great arteries after atrial switch procedure and 8 with congenitally corrected transposition of the great arteries; 5 (22%) females; mean age 38 ± 11 years) were included. Seven (30%) patients had SRV ischemia on SPECT. Late gadolinium enhancement on CMR was more common in patients with SRV ischemia (p = 0.002). However, there was no association between SRV ischemia and different echocardiographic or CMR parameters of SRV systolic function, laboratory markers (high-sensitivity troponin I and NT-proBNP) and exercise capacity. CONCLUSIONS: Our multimodality study showed that SRV ischemia in adult SRV patients was associated with more focal myocardial fibrosis, but not with functional or imaging markers of SRV function.

Adrenal vein sampling for primary aldosteronism: a 15-year national referral center experience.

Background Adrenal vein sampling (AVS) is essential for diagnostics of primary aldosteronism, distinguishing unilateral from bilateral disease and determining treatment options. We reviewed the performance of AVS for primary aldosteronism at our center during first 15 years, comparing the initial period to the period after the introduction of a dedicated radiologist. Additionally, AVS outcomes were checked against CT findings and the proportion of operated patients with proven unilateral disease was estimated. Patients and methods A retrospective cross-sectional study conducted at the national endocrine referral center included all patients with primary aldosteronism who underwent AVS after its introduction in 2004 until the end of 2018. AVS was performed sequentially during Synacthen infusion. When the ratio of cortisol concentrations from adrenal vein and inferior vena cava was at least 5, AVS was considered successful. Results Data from 235 patients were examined (168 men; age 32-73, median 56 years; BMI 18-48, median 30.4 kg/ m2). Average number of annual AVS procedures increased from 7 in the 2004-2011 period to 29 in the 2012-2018 period (p < 0.001). AVS had to be repeated in 10% of procedures; it was successful in 77% of procedures and 86% of patients. The proportion of patients with successful AVS (92% in 2012-2018 vs. 66% in 2004-2011, p < 0.001) and of successful AVS procedures (82% vs. 61%, p < 0.001) was statistically significantly higher in the recent period. Conclusions Number of AVS procedures and success rate at our center increased over time. Introduction of a dedicated radiologist and technical advance expanded and improved the AVS practice.

Data highlighting effects of Ketogenic diet on cardiomyopathy and hepatopathy in Glycogen storage disease Type IIIA.

Datasets highlighting effects of ketogenic diet (KD) in a glycogen storage disease type IIIa patient is presented with the longest patient follow up report to date. Now a 15-year old girl with GSD type IIIa, diagnosed at 1 year of age, had initially introduced treatment with diet high carbohydrates, according to the recommendations. Progressively she developed left ventricular obstructive hypertrophy, hepatomegaly and skeletal myopathy. At the age of 11 years, she was introduced KD and continuous ketosis has been maintained for over 4 years providing longest reported follow up to date. KD introduction lead to a normalization of left ventricular parameters and ventricular mass and to an improvement in hepatic injury markers and decrease in liver size. We provided a table with biochemical parameters, a table providing detailed diet composition, tables with cardiac and hepatic measures and figures depicting cardiac NMR images; all the tables/figures are provided referring to the KD introduction (values prior/after). Interpretation of this data can be found in a case report article titled "Normalization of obstructive cardiomyopathy and improvement of hepatopathy on ketogenic diet in patient with glycogen storage disease (GSD) type IIIa".

Normalization of obstructive cardiomyopathy and improvement of hepatopathy on ketogenic diet in patient with glycogen storage disease (GSD) type IIIa.

Now 15-year-old girl with glycogen storage disease (GSD) type IIIa (OMIM 232400) developed severe left ventricular obstructive hypertrophy and hepatomegaly while treated with frequent cornstarch meals. Subsequently, she was introduced the ketogenic diet; continuous ketosis has been maintained for over the last 4 years. After the introduction of ketogenic diet, a normalization of the cardiomyopathy and improvement of hepatopathy was achieved, with enhanced overall quality of life.

Stent graft implantation in an aortic pseudoaneurysm associated with a fractured Cheatham-Platinum stent in aortic coarctation.

We report a case of aortic pseudoaneurysm associated with a fractured bare Cheatham-Platinum stent following stenting for aortic coarctation. These complications were recognised 6 years after the implantation procedure and were successfully managed by percutaneous stent graft implantation. Staged approach for stent dilatation might prevent development of aortic pseudoaneurysms. In addition, careful follow-up is warranted after stenting for aortic coarctation, particularly in patients with recognised aortic wall injury.

A 6-year follow-up study of adult patients with congenitally corrected transposition.

The aims of this study were to assess the development of heart failure in patients with congenitally corrected transposition of the great arteries in a medium-term follow-up, to identify the impact of tricuspid regurgitation on the development of heart failure, and to determine the most reliable marker for its identification. The prospective 6-year follow-up study included 19 adult patients. All patients were evaluated clinically by the determination of N-terminal pro-hormone brain natriuretic peptide levels, exercise stress testing, echocardiography magnetic resonance, or CT. Among them, two patients died of heart failure. There was a decline in exercise capacity and systolic systemic ventricular function (p=0.011). Systemic ventricular ejection fraction decreased (48.3±13.7 versus 42.7±12.7%, p=0.001). Tissue Doppler imaging showed a decline in peak tricuspid systolic annular velocity (10.3±2.0 versus 8.3±2.5 cm/second, p=0.032) and peak tricuspid early diastolic annular velocity (14.6±4.3 versus 12.0±4.5 cm/second, p=0.048). The tricuspid regurgitation did not increase significantly. N-terminal pro-hormone brain natriuretic peptide levels increased (127.0 ng/L(82.3-305.8) versus 226.0 ng/L(112.5-753.0), p=0.022). Progressive exercise intolerance in congenitally corrected transposition of the great arteries appears to be driven mainly by a progression in systemic right ventricular dysfunction. Tricuspid regurgitation is likely to play a role, especially in patients with structural abnormalities of the tricuspid valve - Ebstein anomaly. The N-terminal pro-hormone brain natriuretic peptide levels and tissue Doppler parameters appear sensitive in detecting changes over time and may guide management.

Aortic valve regurgitation following percutaneous closure of patent ductus arteriosus.

OBJECTIVES: To determine the incidence and outcome of aortic valve regurgitation (AR) following the percutaneous closure of patent ductus arteriosus (PDA). BACKGROUND: Aortic valve regurgitation is an overlooked consequence of percutaneous PDA closure. METHODS: Between December 2000 and May 2009, 51 children underwent percutaneous closure of PDA using Amplatzer Duct Occluders. Their median age at the time of the procedure was 2.6 years (range: 0.6-18.0 years), and median weight was 14.0 kg (range: 7.6-75.0 kg). Follow-up echocardiograms were performed a day after the procedure and at 1, 3, 12 months, and yearly thereafter. RESULTS: A day after the procedure, AR was detected for the first time in 13 of 48 patients (27.0%). A group of patients with newly developed AR was significantly different from a group of patients with competent aortic valves with respect to their age, weight, and minimal PDA diameter indexed to the body weight. The follow-up period ranged from 0.2 to 8.5 years (median 3.3 years) and at the latest follow-up evaluation, AR persisted in a single patient (2.0%) 6 years after the procedure. CONCLUSIONS: The aortic valve regurgitation following percutaneous PDA closure is trivial to mild and transient. It develops in approximately a quarter of children after percutaneous closure of PDA with a minimal diameter≥1.5 mm and is more likely to develop in infants and small children having significant left-to-right shunts.

Nonischemic ST segment elevation in hypertrophic cardiomyopathy due to chest wall deformity from kyphoscoliosis.

A 57-year-old male was admitted with suspected acute coronary syndrome. He reported experiencing moderate chest pain when walking during the day prior to admission, but had very prominent ST segment elevations in the precordial electrocardiography (EKG) leads. A physical examination revealed remarkable severe kyphoscoliosis with chest deformity. The patient's cardiac troponin levels remained normal, while cardiac ultrasound and magnetic resonance imaging of the chest confirmed hypertrophic cardiomyopathy (HCM) with severe thickening of the interventricular septum. Ischemic heart disease was ruled out by myocardial perfusion imaging with (99m)Tc-MIBI during rest and dipyridamole-induced stress without showing irreversible or reversible myocardial ischemia. Our diagnosis was that the chest pain was noncardiac in origin and that the pronounced ST segment elevations in the precordial EKG leads reflected the severely hypertrophic interventricular septum through the normally thick left ventricular free wall. The patient's chest wall deformity brought his septum and the ventricular free wall nearly parallel to the left side of the chest wall, allowing for complete expression of the reciprocal EKG pattern of septal hypertrophy. We suggest that EKG findings should always be interpreted with the chest wall shape being kept in mind.

Balloon dilation of neonatal critical aortic valvar stenosis via the umbilical artery.

OBJECTIVES: To report our experience with balloon dilation of critical aortic valvar stenosis in neonates via the umbilical artery using currently available catheters. BACKGROUND: There is no agreement regarding the optimal vascular approach for balloon dilation of critical aortic valvar stenosis in neonates. METHODS: Since June of 2005, we have attempted to obtain umbilical arterial access within the first week after birth in all neonates with critical aortic valvar stenosis. In patients in whom umbilical artery access was obtained, we proceeded with an attempt at balloon dilation. RESULTS: We were presented with 5 patients with critical aortic valvar stenosis within the first week after birth, and the umbilical arterial approach was obtained in all, with effective relief of the stenosis achieved in 4. CONCLUSIONS: The umbilical arterial approach should always be considered for balloon dilation of neonatal critical aortic valvar stenosis. Using currently available catheters, the procedure is safe, simple, and effective even in patients weighing less than 2.5 kilograms. Further experience using this approach is warranted.

The syndrome of cardiac failure in adults with congenitally corrected transposition.

OBJECTIVES: To assess neurohormonal activation of cardiac failure in adults with congenitally corrected transposition, and to determine the most sensitive marker for recognition of the cardiac failure. BACKGROUND: The onset of morphologically right ventricular dysfunction is unpredictable in patients with congenitally corrected transposition, the combination of discordant atrioventricular and ventriculo-arterial connections, and its markers are unknown. METHODS: We measured amino terminal pro brain natriuretic peptide in 19 patients, aged 35 plus or minus 13.1 years, and in 19 control subjects. Morhologically right ventricular function was assessed by echocardiography, including tissue Doppler echocardiography and magnetic resonance imaging or multislice computed tomography. RESULTS: The patients showed a highly significant increase in the levels of amino terminal pro brain natriuretic peptide, the levels being significantly elevated even in asymptomatic patients. Left atrial dimensions were larger in patients, and significantly lower tissue Doppler echocardiographic velocities were measured at the lateral site of the tricuspid annulus and at the basal segment of the interventricular septum. The ejection fraction of the morphologically right ventricle correlated significantly with the levels of brain natriuretic peptide, and with left atrial dimensions. CONCLUSIONS: Neurohormonal activation is present in patients with congenitally corrected transposition even when they are asymptomatic. It is correlated with left atrial dimensions and tissue Doppler echocardiographic parameters. Levels of brain natriuretic peptide, and peak tricuspid early diastolic annular velocity, are the earliest and most sensitive markers of morphologically right ventricular dysfunction.

Percutaneous management of cyanosis in Fontan patients using Amplatzer occluders.

OBJECTIVES: To determine causes of cyanosis and to evaluate percutaneous management of cyanosis in a group of consecutive Fontan patients. BACKGROUND: A variety of communications allow a right-to-left shunt in Fontan circulation causing cyanosis and these communications are amenable to percutaneous closure. METHODS: Between November 1997 and November 2007, 45 consecutive patients ranging in age from 2.5 to 26 years (median 8 years) with Fontan circulation and cyanosis underwent cardiac catheterization and percutaneous closure of superfluous communications using different types of Amplatzer occluders. RESULTS: Altogether, 51 communications were detected and 50 of them were closed. In 5 patients (11.1%), multiple communications were present and were closed. Fenestration was detected in 37 of 41 patients (90.2%) after total cavopulmonary connection and all were closed by Amplatzer septal occluders. Five venous collaterals were revealed in 3 of 41 patients (7.3%) with total cavopulmonary anastomosis and were closed by Amplatzer vascular plugs. In 3 patients, lateral tunnel leaks were detected and were closed by Amplatzer PFO occluders. In 4 patients after Kawashima operation, 3 major pulmonary arterio-venous malformations and single venous collateral contributed to the cyanosis and all were closed using Amplatzer vascular plugs. CONCLUSIONS: Different communications cause cyanosis in Fontan patients. Progressive decline of percutaneous oxygen saturation is suggestive of development of venous collaterals or pulmonary arteriovenous malformations. Superfluous communications are amenable to percutaneous closure using various types of Amplatzer occluders. A novel use of an Amplatzer PFO occluder for the percutaneous closure of a lateral tunnel leak is described.