Oxygen inhalation decreases the central venous pressure in adult patients late after Fontan operations.

BACKGROUND: Elevated central venous pressure (CVP) and decreased arterial oxygen saturation (SaO2) are the characteristics of patients after Fontan operations and determine morbidity and mortality in the long-term. Oxygen inhalation therapy theoretically increases SaO2 and may decrease the elevated CVP in these patients. However, there is no previous study to support this hypothesis. This study aimed to determine the acute effects of oxygen inhalation on the hemodynamics of adult patients late after Fontan operations using cardiac catheterization. METHODS: This study enrolled 58 consecutive adult patients (median age, 30 years; female, n = 24) who had undergone Fontan operations. We assessed the hemodynamic changes during oxygen inhalation (2 L/min) with a nasal cannula in cardiac catheterization. We divided the studied patients into two groups according to the reduction in CVP during oxygen inhalation using the median value: responders (>2 mmHg) and non-responders (≤2 mmHg). Clinical characteristics of the responders to oxygen inhalation were investigated with uni- and multivariate analyses. RESULTS: SaO2 increased from 93.3 % (91.3-94.5 %) to 97.5 % (95.2-98.4 %) (p < 0.001) and CVP decreased from 12 mmHg (11-14 mmHg) to 10 mmHg (9-12 mmHg) (p < 0.001) after oxygen inhalation. There was a weak but significant correlation between the increase in SaO2 and the decrease in CVP (R = 0.29, p = 0.025). Pulmonary blood flow increased from 4.1 L/min (3.5-5.0 L/min) to 4.4 L/min (3.7-5.3 L/min) (p = 0.007), while systemic blood flow showed no significant changes. A multivariate analysis revealed that high baseline CVP was associated with a larger decrease in CVP (>2 mmHg) after oxygen inhalation. CONCLUSIONS: Oxygen inhalation increased SaO2 and decreased CVP, especially in patients with high baseline CVP. Further studies with home oxygen therapy are needed to investigate the long-term effects of oxygen inhalation in adult patients who underwent Fontan operations.

Therapeutic Response to Single-Inhaler Triple Therapies in Moderate-to-Severe COPD.

BACKGROUND: COPD is characterized by progressive and irreversible air flow limitations. Single-inhaler therapies (SITTs) incorporating an inhaled corticosteroid, a long-acting muscarinic antagonist, and a long-acting ß2-agonist have been shown to effectively alleviate symptoms and improve lung function. Fluticasone-furoate/umeclidinium/vilanterol (F/U/V) and budesonide/glycopyrronium/formoterol (B/G/F) are available as SITT in Japan. However, the clinical differences between these 2 combinations and the predictors of their proper use have not been established. This study aimed to identify the subject characteristics that could predict the effectiveness of inhaler therapy. METHODS: We assessed the pulmonary function test results of subjects with COPD before and one month after using F/U/V and B/G/F as SITT. Subjects with a difference of 100 mL or more in the FEV1 after treatment with pre-SITT were extracted and divided into the F/U/V effect and no-effect group and B/G/F effect and no-effect group to examine the factors associated with positive outcomes with each inhaler. RESULTS: F/U/V and B/G/F significantly improved the inspiratory capacity (IC), %IC, FVC, and %FEV1 when compared to pre-intervention values (P < .001, P = .001, P = .007, P = .009, respectively, for F/U/V; and P = .006, P = .008, P = .038, P = .005, respectively, for B/G/F). Factors associated with FEV1 improvement in F/U/V included lower %IC (odds ratio 0.97 [95% CI 0.94-0.99], P = .03) and a higher modified Medical Research Council (mMRC) dyspnea score (2.36 [1.27-4.70], P < .01). In addition, a higher %IC (1.03 [1.00-1.06], P = .02) and lower mMRC dyspnea score (0.55 [0.28-0.99], P = .041) were predictors for the effectiveness of B/G/F. CONCLUSIONS: Our results showed that SITT significantly improved the IC, %IC, FVC, and %FEV1 when compared to pre-intervention and that F/U/V was more effective in subjects with severe symptoms, whereas B/G/F was more effective in subjects with mild symptoms.

High-echoic line tracing of transthoracic echocardiography accurately assesses right ventricular enlargement in adult patients with atrial septal defect.

Accurate measurement of right ventricular (RV) size using transthoracic echocardiography (TTE) is important for evaluating the severity of congenital heart diseases. The RV end-diastolic area index (RVEDAi) determined using TTE is used to assess RV dilatation; however, the tracing line of the RVEDAi has not been clearly defined by the guidelines. This study aimed to determine the exact tracing method for RVEDAi using TTE. We retrospectively studied 107 patients with atrial septal defects who underwent cardiac magnetic resonance imaging (CMR) and TTE. We measured the RVEDAi according to isoechoic and high-echoic lines, and compared it with the RVEDAi measured using CMR. The isoechoic line was defined as the isoechoic endocardial border of the RV free wall, whereas the high-echoic line was defined as the high-echoic endocardial border of the RV free wall more outside than the isoechoic line. RVEDAi measured using high-echoic line (high-RVEDAi) was more accurately related to RVEDAi measured using CMR than that measured using isoechoic line (iso-RVEDAi). The difference in the high-RVEDAi was 0.3 cm2/m2, and the limit of agreement (LOA) was - 3.7 to 4.3 cm2/m2. With regard to inter-observer variability, high-RVEDAi was superior to iso-RVEDAi. High-RVEDAi had greater agreement with CMR-RVEDAi than with iso-RVEDAi. High-RVEDAi can become the standard measurement of RV size using two-dimensional TTE.

Visibility of Pulmonary Valve and Pulmonary Regurgitation on Intracardiac Echocardiography in Adult Patients with Tetralogy of Fallot.

Pulmonary regurgitation (PR) is a risk factor for sudden cardiac death in adult patients with repaired tetralogy of Fallot (TOF). However, transthoracic echocardiography (TTE) cannot fully visualize the pulmonary valve (PV) and PR. We investigated whether intracardiac echocardiography (ICE) could visualize the PV and PR better than TTE. Thirty adult patients with TOF (mean age 33 ± 15 years) scheduled for cardiac catheterization underwent ICE. The visualization of PV and the severity of PR were classified into three grades. ICE depicted the PV better than TTE (ICE vs. TTE: not visualized, partially visualized, and fully visualized: n = 1 [3%], n = 13 [43%], and n = 16 [53%] vs. n = 14 [47%], n = 13 [43%], and n = 3 [10%], p < 0.001). Especially in patients after pulmonary valve replacement (PVR), the PV was more fully visualized by ICE. The assessment of PR by TTE underestimated the severity of PR in comparison to cardiac magnetic resonance imaging (MRI) (severe PR: 8 [28%] vs. 22 [76%], p = 0.004), while there was no discrepancy between the results of ICE and MRI (21 [72%] vs. 22 [76%], p = 1.000). In comparison to TTE, ICE can safely provide better visualization of the PV and PR in adults with TOF, especially in patients who have undergone PVR.

Detection of a unidirectional epicardial connection between the right-sided pulmonary venous carina and the right atrium by pacing from a high-density mapping catheter.

We report a case of recurring, persistent atrial fibrillation (AF) in a patient with a unidirectional epicardial connection (EC) between the right-sided pulmonary venous (PV) carina and the right atrium detected using a high-density mapping catheter with a steerable introducer support, but not a conventional circular mapping catheter. This unidirectional EC could be steadily abolished by a radiofrequency delivery. Finally, we were able to successfully achieve complete PV antrum isolation. Thereafter, he has remained well without any AF.

Successful Total Management of Multi-Causative Sleep-Disordered Breathing Complicated with Patient with Adult Congenital Heart Disease.

Sleep-disordered breathing is one of the complications commonly seen in patients with adult congenital heart disease (ACHD) due to multiple causes including complex underlying cardiac defects, cardiomegaly, previous thoracotomies, obesity, scoliosis, and paralysis of the diaphragm. It is often hard to determine its main cause and predict the efficacy of each treatment in its management. We herein report a 30-year-old woman after biventricular repair of pulmonary atresia with intact ventricular septum diagnosed as sleep-related hypoventilation disorder. Simultaneous treatment targeting obesity, paralysis of the diaphragm, and cardiomegaly followed by respiratory muscle reinforcement through non-invasive ventilation resolved her sleep-related hypoventilation disorder. Such management for each factor responsible for the hypoventilation is expected to provide synergetic therapeutic efficacy and increase daily activity in a patient with ACHD.

Epicardial Connections After a Conventional Pulmonary Vein Antrum Isolation in Patients With Atrial Fibrillation.

BACKGROUND: The existence of epicardial connection(s) (ECs) between the pulmonary veins (PVs) and atrium may hinder establishing a complete PV antrum isolation (AI) (PVAI) in patients with atrial fibrillation (AF). Thus, the purpose of this study was to determine the prevalence and location of ECs inside the conventional PVAI lines.Methods and Results: Three-hundred consecutive patients with non-valvular AF were evaluated. This study revealed that: (1) the prevalence of patients with ECs and the number of ECs per patient between the PVs and atrium became significantly greater, respectively, in accordance with the progression of paroxysmal to long-lasting AF and left atrial enlargement; (2) some ECs were located at sites far distal to the PVAI lines; (3) 25% of ECs could be detected only by high-density mapping catheters, but not by conventional circular mapping catheters; (4) a B-type natriuretic peptide (BNP) level of 176.6pg/mL and left atrial volume (LAV) of 129.0 mL may be important predictors of the presence of ECs; and (5) the rate of conduction of ECs from the right PVs was dominantly to the atrium and His-bundle, and that from the left PVs to the coronary sinus was most dominant. CONCLUSIONS: The PVAI may not be completed by using only a conventional PVAI method, and additional EC ablation inside the PVAI lines detected using high-density mapping may be able to achieve a more complete PVAI.

Hemodynamic Characteristics After Fontan Procedure in Patients with Down's Syndrome.

Patients with Down's syndrome (DS) are generally regarded as not being good candidates for the Fontan procedure. However, detailed hemodynamic changes over time are not fully clarified. A retrospective chart review of all patients with DS who underwent the Fontan procedure and 5 times that number of Fontan patients without DS performed in Fukuoka Children's Hospital and Kyushu University Hospital. Seven Fontan patients with DS were identified, and 35 Fontan patients without DS were recruited. During the mean observational periods of 14.7 years and 15.0 years (DS and non-DS, respectively) after the Fontan procedure, only one DS patient died. Central venous pressure (CVP) and transpulmonary pressure gradient significantly increased, and arterial oxygen saturation significantly decreased over time in DS patients after the Fontan procedure compared with those without DS. CVP in DS patients after the Fontan procedure increased over time compared with non-DS patients. Better management including the efficacy of Pulmonary arterial hypertension-specific therapy should be clarified in further studies.

Increased Pulmonary Arterial Compliance after Balloon Pulmonary Angioplasty Predicts Exercise Tolerance Improvement in Inoperable CTEPH Patients with Lower Pulmonary Arterial Pressure.

BACKGROUND: Balloon pulmonary angioplasty (BPA) improved pulmonary arterial compliance (CPA) and exercise tolerance in patients with inoperable chronic thromboembolic pulmonary hypertension (CTEPH). OBJECTIVES: To investigate whether CPA is a useful index to indicate exercise tolerance improvement by BPA in CTEPH patients. METHODS: The correlation between changes in CPA and improvements in 6-minute walk distance (6MWD) by BPA was retrospectively analyzed in 70 patients (Analysis 1), and it was sequentially analyzed in 46 symptomatic patients who achieved mean pulmonary arterial pressure (mPAP)<30mmHg (Analysis 2). RESULTS: We enrolled 70 patients (female/male:57/13, mean age:59 years) who underwent a total of 352 BPA sessions which significantly increased CPA (1.5±0.8 vs. 3.0±1.0 mL/mmHg) and decreased pulmonary vascular resistance (PVR) (8.0 ± 3.9 vs. 3.6 ± 1.7 wood units). The correlation coefficient between improvement in 6MWD and changes in PVR and CPA were r=0.21 (p=0.09) and r=0.14 (p=0.26) (Analysis 1). In Analysis 2, those were r=0.32 (p=0.06) and r=0.38 (p=0.02), respectively. CONCLUSIONS: CPA can be a useful index to indicate the improvement in exercise tolerance by BPA in symptomatic patients with lower mPAP.

Usefulness of 18F-Fluorodeoxyglucose Positron Emission Tomography/Computed Tomography in the Diagnosis of Infective Endocarditis in Patients With Adult Congenital Heart Disease.

BACKGROUND: Infective endocarditis (IE) in patients with adult congenital heart disease (ACHD) remains a diagnostic challenge due to difficulties in detecting endocardial lesions by echocardiography. 18F-fluorodeoxyglucose (18F-FDG) positron emission tomography/computed tomography (PET/CT) has shown good diagnostic performance in prosthetic valve IE. This study aimed to assess its additional diagnostic value in ACHD-associated IE and to characterize its advantages.Methods and Results:Overall, 22 patients with ACHD and clinical suspicion of IE were retrospectively studied. 18F-FDG PET/CT was performed in addition to conventional assessment based on the modified Duke criteria. The final IE diagnosis was determined by an expert team during a 3-month clinical course, resulting in 18 patients diagnosed with IE. Seven patients (39%) were diagnosed with definite IE only by initial echocardiography. An 18F-FDG PET/CT assessment revealed endocardial involvement in the other 9 patients, resulting in the diagnosis of definite IE in 16 in total (88%). Right-sided endocardial lesions were more common (n=12, 67%) but rarely identified by echocardiography, whereas 18F-FDG PET/CT revealed right-sided lesions in 9 patients. A negative 18F-FDG PET/CT (n=7, 39%) assessment was associated with a native valve IE (71% vs. 0%). In 4 patients who were identified with not-IE, neither echocardiography nor 18F-FDG PET/CT detected any suspicious cardiac involvement. CONCLUSIONS: In the diagnosis of ACHD-associated IE, characterized by right-sided IE, 18F-FDG PET/CT assessment should be useful.

Preoperative Threshold for Normalizing Right Ventricular Volume After Transcatheter Closure of Adult Atrial Septal Defect.

BACKGROUND: The latest guidelines recommend early intervention in adult atrial septal defect (ASD) patients with signs of right ventricular (RV) enlargement. However, the criteria of RV enlargement for optimal intervention remain unclear. We investigated the preoperative determinants for normalizing the RV volume after transcatheter closure of ASD in adults.Methods and Results:We retrospectively analyzed 52 ASD patients who underwent transcatheter closure. Cardiac magnetic resonance imaging (CMR) measured RV volume before and 1 year after the closure. The patients were divided into normalized (postoperative RV end-systolic volume index [RVESVI] <47 mL/m2and end-diastolic volume index [RVEDVI] <108 mL/m2) and non-normalized (postoperative RVESVI ≥47 mL/m2or RVEDVI ≥108 mL/m2) groups. Preoperative RVESVI was significantly smaller (72 mL/m2vs. 80 mL/m2) and RVEF was higher (56% vs. 51%) in the normalized group compared with the non-normalized group. Receiver-operating characteristic analysis for the normalization of postoperative RV volume showed that the preoperative threshold value of RVESVI was 75 mL/m2. In addition, multivariate analysis showed that preoperative RVESVI was an independent predictor for normalization of RV volume. CONCLUSIONS: Preoperative RVESVI is an independent predictor for normalization of RV volume at 1 year after transcatheter closure of ASD in adults. Early intervention before RVESVI reaches 75 mL/m2may confer optimal timing for normalizing RV volume.

Elaborate evaluation of serial changes in electrocardiograms of atrial septal defects after transcatheter closure for a better understanding of the recovery process.

Serial changes of electrocardiograms (ECG) could be used to assess their clinical features in atrial septal defects (ASD) after transcatheter closure together with other clinical parameters. We retrospectively studied 100 ASD patients who underwent transcatheter closure. Complications of persistent atrial fibrillation occurred in five ASD patients, and they were excluded. We divided the other 95 patients according to PQ intervals before closure (normal: < 200 ms, n = 51; prolonged: ≥ 200 ms, n = 44) to evaluate their clinical characteristics and parameters such as echocardiography, chest X-rays, and brain natriuretic protein (BNP) levels. Individuals in the prolonged PQ group were significantly older, had higher incidences of paroxysmal atrial fibrillation (PAF) and heart failure (HF) treated with more ß-blockers and diuretics, and with a higher tendency of NYHA functional classification and BNP levels than the normal PQ group. The prolonged PQ group also had a significantly higher incidence of complete right bundle branch block, wider QRS intervals, and larger cardiothoracic ratios in chest X-rays accompanied by larger right atrial-areas and larger left atrial dimensions in echocardiograms. Furthermore, the prolonged PQ intervals with less PQ interval shortening after transcatheter closure revealed that the patients were the oldest at the time of closures and showed less structural normalization of the right heart and left atrium after ASD closure. PAF and HF also occurred more frequently in this subgroup. These results suggested that the ASD patients with prolonged PQ intervals with less PQ shortening were accompanied by more advanced clinical conditions. Together with other clinical parameters, detailed analyses of ECG and their changes after closure could elucidate the clinical characteristics and status of ASD patients with transcatheter closure and were useful for predicting structural normalization after transcatheter closure.

Outcome of patients with functional single ventricular heart after pacemaker implantation: What makes it poor, and what can we do?

BACKGROUND: Pacemaker implantation in patients with single ventricle is associated with poor outcomes. OBJECTIVE: The purpose of this study was to determine the reasons for the poor outcomes of pacemaker implantation. METHODS: We performed a retrospective chart review of patients with single ventricle who had undergone permanent pacemaker implantation. Patients were categorized into 3 groups based on the site of pacing and the proportion of ventricular pacing (VP) as follows: (1) atrial pacing group with atrial pacing only (n = 11); (2) low VP group with low daily VP proportion (<50%; n = 12); and (3) high VP group with high daily VP proportion (≥50%; n = 15). Pacing leads were placed at the epicardium in all patients. RESULTS: No patients in the atrial pacing or low VP groups died, whereas the survival rate in the high VP group was 58.9% and 39.3% at 10 and 20 years, respectively, after pacemaker implantation. Among the post-Fontan patients, plasma brain natriuretic peptide (BNP) levels significantly increased with the proportion of VP: 11.7, 20.3, and 28.4 pg/mL in the atrial pacing, low VP, and high VP groups, respectively (P = 0.04). In the high VP group, the plasma BNP level was significantly lower in patients with an apical pacing lead than in those with a nonapical pacing lead (27.0 pg/mL vs 82.8 pg/mL, respectively; P = .03). CONCLUSION: A higher proportion of VP was associated with poor outcome and higher plasma BNP levels, probably due to ventricular dyssynchrony. In epicardial ventricular pacing, apical pacing is better to avoid the increase in ventricular stress and plasma BNP level.

Blackout during meals: A case report of swallow syncope due to sinus arrest.

A 79-year-old male, with a history of percutaneous coronary intervention (PCI), was referred to our cardiovascular department for a detailed examination of blackout caused by sinus arrest only during meals. Ultrasound echocardiography showed normal cardiac contraction with no asynergy, irrespective of the remaining stenotic coronary lesion. An electrophysiological study revealed deteriorated atrioventricular nodal conduction at a Wenckebach point of 70 beats per minute. However, sinus node function was normal as demonstrated by a sinus node recovery time of 1369 ms. Coronary angiography showed triple-vessel disease including the remaining stenotic coronary lesion, and a PCI was performed on the right coronary artery. Nevertheless, sinus arrest during meals was unchanged. Swallow syncope was partially improved by dietary modification; however, pacemaker implantation (PMI) was performed eventually, and the patient became asymptomatic after PMI. .