Transcatheter Occlusion of a Giant Coronary Artery Fistula in a Neonate.

BACKGROUND Although large coronary artery fistulas are uncommon; they lead to substantial over-circulation in the pulmonary vascular beds and left heart system. Fistula occlusions are achieved via surgical or transcatheter technique; however, reports on successful outcomes of transcatheter treatment during the neonatal period are limited. CASE REPORT A female infant was born at the gestational age of 37 weeks with a birth weight of 2615 grams via normal vaginal delivery. Cardiac auscultation revealed a loud continuous murmur emanating from the fourth right intercostal space. A right coronary artery-to-right ventricle fistula was confirmed using transthoracic echocardiography. The newborn developed respiratory distress 3 days after birth and was administered continuous positive airway pressure to assist breathing. On day 8, the ventilator was used through tracheal intubation due to gradual worsening of dyspnea. A 6-mm Amplatzer Vascular Plug 4 (AGA Medical Corporation, Plymouth, MN) was chosen, as the minimum diameter of the coronary artery fistula was 5 mm. In view of the risk of myocardial ischemia with additional devices, the procedure was stopped despite persistent shunting. The newborn's clinical condition significantly improved following the procedure and she was eventually weaned off ventilator support. CONCLUSIONS A self-expanding occlusion device was useful for relieving this life-threatening condition. Complete elimination of shunting is not always necessary, to avoid compromising myocardial circulation.

A Japanese Patient with Genitopatellar Syndrome Transiently Presenting with Cardiac Intramural Cavity during the Neonatal Period.

Genitopatellar syndrome (GPS) is a rare autosomal dominant disorder caused by de novo pathogenic variants in the KAT6B gene. It is characterized by genital abnormalities, patellar hypoplasia/agenesis, flexion contractures of the hips and knees, corpus callosum agenesis with microcephaly, and hydronephrosis and/or multiple renal cysts. More than half of patients with GPS have congenital heart defects, mostly atrial and/or ventricular septal defects, patent foramen ovale, and patent ductus arteriosus. We report a case of a Japanese neonate with a de novo heterozygous c.3769_3772delTCTA pathogenic variant in the KAT6B gene who presented with a cardiac intramural cavity of the ventricular septum at birth. The cavity unexpectedly disappeared at 1 month of age, but trabecular septal thinning and flash remained. The features of the cavity were not consistent with those of congenital ventricular diverticulum or aneurysm, and its identity and prognosis are still unclear. Because patients with GPS may exhibit various forms of cardiac malformation, careful cardiac examination and follow-up are required from birth in cases of suspected GPS.

Development of suction force during early diastole from the left atrium to the left ventricle in infants, children, and adolescents.

Although the suction force that moves blood into the left ventricle during early diastole is thought to play an important role in diastolic function, there have been a few studies of this phenomenon in normal children. Suction force is measured as the intraventricular pressure difference (IVPD) and intraventricular pressure gradient (IVPG), which is calculated as IVPD divided by left ventricular length. The purpose of this study was to determine the suction force in infants, children, and adolescents using IVPD and IVPG. We included 120 normal children categorized into five groups based on age: G1 (0-2 years), G2 (3-5 years), G3 (6-8 years), G4 (9-11 years), and G5 (12-16 years). The total, basal, and mid-apical IVPD and IVPG were calculated using color M-mode Doppler imaging of the mitral valve inflow using the Euler equation. The total IVPD increased with age from G1 to G5 (1.75 + 0.51 vs. 2.95 + 0.72 mmHg, respectively; p < 0.001), due to an increase in mid-apical IVPD with constant basal IVPD. Although total IVPG was constant, mid-apical IVPG was larger in G5 than in G1 (0.21 + 0.06 vs. 0.16 + 0.07 mmHg/cm, respectively; p = 0.006). Total, basal, and mid-apical IVPDs were significantly correlated with age and the parameters of heart size and mitral annular e'. Mid-apical IVPG correlated with age and e' positively, but basal IVPG did with age negatively and did not with e'. The suction force increased at the mid-apical segment, correlating with increasing heart size and developing left ventricular relaxation, even after adjustment for left ventricular length.

Measurement of Aortic Valve Coaptation and Effective Height Using Echocardiography in Patients with Ventricular Septal Defects and Aortic Valve Prolapse.

Decreased coaptation height in adults has been identified as a marker of early valve failure. We evaluated aortic valve coaptation and effective height in healthy children and in children with a ventricular septal defect (VSD) with aortic cusp prolapse (ACP), using echocardiography. We included 45 subjects with VSD with ACP, 27 did not develop aortic regurgitation (AR) by ACP and 18 developed AR by ACP, and 83 healthy children as controls. Aortic root anatomy was estimated using the parasternal long-axis view. We measured the diameter of aortic valve (AV), coaptation height (CH), and effective height (EH) of the aortic valve. We defined the ACH (CH/AV ratio) and AEH (EH/AV ratio) indices as follows: [Formula: see text]. There were significant differences in ACH and AEH between the groups (control vs VSD with ACP vs VSD with ACP and AR, median ACH [%], 35.1 vs 32.0 vs 22.1; median AEH [%], 52.0 vs 48.0 vs 34.4, respectively; P < 0.01]). Intra-cardiac repair (ICR) was performed in 15 cases. Significant increases were observed in ACH and AEH before and after ICR (median ACH [%], before: 27.0, after: 32.7, P < 0.05; median AEH (%), before 38.5, after 45.8, P < 0.05). Measurement of ACH and AEH may allow direct and non-invasive assessment of the severity of VSD with ACP, which could aid clinicians in determining the need and timing for surgical intervention.

Assessment of the developmental change in the left atrial volume using real time three-dimensional echocardiography.

AIMS: Real time 3D echocardiography (RT3DE) has been applied for the assessment of left atrial (LA) function in patients with adult heart disease; however, LA function is not well known in children. We aimed at determining the normal range of LA volume (LAV) using RT3DE and the feasibility and reproducibility of this method in healthy subjects and at elucidating the developmental changes in the LAV with aging. METHODS AND RESULTS: In this study, 359 healthy people (mean age, 23.9 ± 21.3; range, 0.1-76.4 years) were enrolled. We performed transthoracic RT3DE and measured the maximum and minimum LAV. Simultaneously, we measured the LAV using the 2D biplane Simpson's method. Inter-observer and intra-observer variability and the agreement of LAV measurements between RT3DE and 2DE were assessed in a subset of subjects. The RT3DE feasibility for LAV measurement was 93%. Both maximum and minimum LAVs exponentially increased with age and linearly increased with increasing of body surface area (BSA). The LA distensibility, which demonstrates LA reservoir function, decreased with age and BSA. The LAVs measured by RT3DE were significantly smaller than those measured by the 2D biplane Simpson's method. The 3D volumetric method had favorable intra-observer and inter-observer agreement. CONCLUSION: The reference values of LAV from early infancy to adulthood were obtained using RT3DE, which could be useful for future studies in children with congenital heart disease. RT3DE is a reproducible method and a feasible tool for evaluating the LAV in children. LA reservoir function is likely to decrease with age and increasing of body size.

Altered central aortic elastic properties in Kawasaki disease are related to changes in left ventricular geometry and coronary artery aneurysm formation.

BACKGROUND: Recent evidence has suggested that patients display altered arterial elasticity after Kawasaki disease (KD). However, changes in the elastic properties of the central aorta and their relevance to left ventricular geometry have not been studied in patients after KD with and without coronary artery aneurysms (CAAs). METHODS: Clinical and laboratory characteristics of 75 patients with KD were compared with those of 57 controls. The patients with KD included 17 patients with CAAs and 58 patients without CAAs. Values for aortic stiffness index, aortic distensibility, aortic strain, and left ventricular mass index (LVMI) were retrospectively obtained from echocardiographic measurements of the ascending aorta and left ventricle with noninvasive blood pressure evaluation. RESULTS: Systolic blood pressure, pulse pressure, LVMI, and aortic stiffness index were significantly higher and aortic distensibility and aortic strain significantly lower in patients with KD than in the controls. In patients with KD, age at the time of study, interval between the onset of KD and the initiation of this study, CAAs, and LVMI were significantly associated with aortic stiffness index, aortic distensibility, and aortic strain. Multivariate analysis revealed that CAAs and LVMI were independently relevant to aortic stiffness index and aortic distensibility. CONCLUSIONS: The central aortas of patients after KD have altered elastic properties. CAAs and LVMI are independently correlated with central aortic elasticity.

Echocardiographic predictors for persistent functional mitral regurgitation after aortic valve replacement in patients with aortic valve stenosis.

Moderate functional mitral regurgitation (MR) in patients with aortic valve stenosis (AS) is often left unaddressed at the time of aortic valve replacement (AVR) because it is expected to decrease after AVR. However, some patients have persistent moderate MR after AVR. We sought to determine the preoperative echocardiographic predictor for persistent functional MR after AVR in patients with AS. Pre- and postoperative echocardiograms were reviewed in 110 patients with severe AS and functional MR who underwent AVR without mitral valve (MV) surgery. Fifty-eight patients received concomitant coronary artery bypass graft surgery. In patients with MV tenting, defined as apical displacement of mitral leaflets in the apical 4-chamber view, MV tenting area and tenting height were measured at midsystole. Eighty patients had MV tenting (mean MV tenting area 1.4 +/- 0.5 cm(2), mean MV tenting height 0.8 +/- 0.2 cm) and 30 did not have it before AVR. MR severity decreased in 51 of 80 patients (64%) with MV tenting after AVR and in 25 of 30 patients (83%) without MV tenting (p <0.05). In patients with MV tenting, multivariate analysis revealed that presence of long-term atrial fibrillation and MV tenting area were independent predictors of postoperative MR severity (all p values <0.05). The sensitivity and specificity in predicting persistent moderate MR after AVR were 72% and 82% for MV tenting area >1.4 cm(2). In conclusion, preoperative MV tenting predicts persistent functional MR after AVR in patients with severe AS.

Long-term administration of beraprost sodium for pulmonary arterial hypertension associated with congenital heart disease.

Pulmonary arterial hypertension (PAH) is often associated with congenital heart disease (CHD). Acute administration of beraprost reduces pulmonary vascular resistance in patients with idiopathic PAH and PAH associated with CHD; however, little is known about whether or not long-term treatment with oral beraprost benefits these patients. We report the case of a patient suffering from severe PAH associated with large patent ductus arteriosus (PDA), who was considered to be ineligible for PDA closure using a conventional treatment strategy. Eventually, long-term administration of oral beraprost ameliorated the degree of PAH and the patient subsequently underwent successful closure of the PDA.

Persistent tricuspid regurgitation and its predictor in adults after percutaneous and isolated surgical closure of secundum atrial septal defect.

The fate of functional tricuspid regurgitation (TR) after closure of a secundum atrial septal defect (ASD) without any corrective tricuspid valve (TV) surgery remains unclear. We investigated this and the predictors of persistent TR after ASD closure. Thirty-two consecutive patients with moderate or severe TR before ASD closure were examined. Of these, 23 underwent percutaneous ASD closure, and 9 underwent isolated surgical ASD closure. The left ventricular end-diastolic volume, left ventricular ejection fraction, right ventricular end-diastolic area, right ventricular fractional area change, right ventricular spherical index, right atrial area, TV annular diameter, TV tethering height, pulmonary artery systolic pressure, and pulmonary/systemic blood flow ratio were determined by echocardiography before and early after ASD closure. The color Doppler maximal jet area was used to assess the severity of TR. After ASD closure, the jet area decreased for all patients (p = 0.009); however, 16 patients (50%) had persistent TR. Multivariate analysis revealed that only pulmonary artery systolic pressure before ASD closure was related to the TR jet area after ASD closure (p = 0.003). A pulmonary artery systolic pressure of >60 mm Hg predicted persistent TR with 100% sensitivity and 63% specificity. In conclusion, functional TR was ameliorated after percutaneous and isolated surgical ASD closure, although persistent TR was common. The presence of pulmonary hypertension before ASD closure predicted persistent TR; therefore, corrective TV surgery should be considered at ASD closure in adult patients with moderate or severe TR and concomitant pulmonary hypertension.

Left atrial function assessed by real-time 3-dimensional echocardiography is related to right ventricular systolic pressure in chronic mitral regurgitation.

BACKGROUND: Left atrial (LA) volume is a prognostic index in chronic mitral regurgitation (MR). However, little is known about LA function in this setting. We hypothesized that LA dysfunction is related to pulmonary hypertension in chronic MR. METHODS: Seventy-one patients with organic chronic MR who underwent real-time 3-dimensional transthoracic echocardiography (RT3DE) were studied. Left atrial volumes and peak passive and active LA emptying rates were obtained. Total LA emptying fraction was calculated as follows: [(maximum - minimum LA volume)/maximum LA volume] x 100. Similarly, active and passive LA emptying fractions were calculated. From transmitral flow, the peak early (E) and late (A) diastolic filling velocities and E/A ratio were obtained. The early (E') and late (A') diastolic myocardial velocities were obtained by tissue Doppler interrogation of mitral annulus. RESULTS: Effective regurgitant orifice area (EROA) was 0.57 +/- 0.29 cm(2). Right ventricular systolic pressure (RVSP) was measured in 57 patients and averaged 37 +/- 13 mm Hg. Patients with MR and high RVSP displayed higher minimum LA volume, E/A ratio, E/E' ratio, EROA, and MR volume, and lower A' velocity, peak active LA emptying rate, active LA emptying fraction, and total LA emptying fraction than patients with MR and normal RVSP. Multiple regression analysis revealed that EROA (r = 0.51, P = .01) active LA emptying fraction (r = -0.53, P = .02), E/E' ratio (r = 0.50; P = .04), and the lateral A' velocity (r = -0.46; P = .003) were independently correlated with RVSP. CONCLUSIONS: Left atrial function determined by RT3DE had significant correlation with RVSP in chronic MR, irrespective of MR severity. Thus, pulmonary hypertension in chronic MR may depend not only on MR severity but also on LA function.

Different determinants of residual tricuspid regurgitation after tricuspid annuloplasty: comparison of atrial septal defect and mitral valve prolapse.

We analyzed 20 patients with atrial septal defect (ASD) who underwent tricuspid valve (TV) annuloplasty and ASD closure, 21 patients with mitral valve prolapse (MVP) who underwent mitral valve (MV) and TV annuloplasty, and 20 healthy controls. Severity of tricuspid regurgitation (TR) was assessed by maximal TR jet area/RA area (%TR) using echocardiography before and early after surgery. Before surgery, 2 groups of patients showed significantly greater RA area, TV annulus diameter, RV systolic pressure, and %TR than controls. %TR was significantly decreased after surgery, whereas residual TR was shown in 19% of the MVP group and 25% of the ASD group. Preoperative TV tethering height and %TR were significantly associated with postoperative %TR in the MVP group, whereas preoperative RV fractional area change, RV spherical index, and RV systolic pressure were significantly associated with postoperative %TR in the ASD group. Risk stratification after TV annuloplasty should take the structural abnormality into consideration.

Doppler-derived preoperative mitral regurgitation volume predicts postoperative left ventricular dysfunction after mitral valve repair.

BACKGROUND: Unexpected postoperative left ventricular (LV) dysfunction after valve repair for mitral regurgitation (MR) occurs in some patients with normal preoperative LV function. Identification of factors that predispose to such LV dysfunction would enhance our understanding of the indications and outcomes of surgery. METHODS: We retrospectively analyzed pre- and postoperative (median fourth day) echocardiograms of 174 patients undergoing valve repair for pure and isolated MR. Preoperative MR volume was quantified by the quantitative Doppler and/or proximal isovelocity surface area method. RESULTS: There was an incremental predictive value of MR quantification over the current recommendations (global chi(2) from 48.14 to 81.57, P < .001; Hosmer-Lemeshow test, P = .98), for postoperative LV dysfunction, defined as ejection fraction <50%. The independent predictors were MR volume and LV end-systolic dimension (P < .001 and P = .01, respectively). Sixty-nine patients underwent surgery before development of the current surgical criteria, namely, symptoms, atrial fibrillation, preoperative LV dysfunction, or pulmonary hypertension. Of these, MR volume was the only independent significant predictor (P < .001) of unexpected postoperative LV dysfunction that developed in 14 patients (20%). Unexpected LV dysfunction could be predicted with sensitivity of 86% (95% CI 67%-100%) and specificity of 89% (95% CI 81%-97%), using the optimal cutoff of 80 mL for MR volume. CONCLUSIONS: Doppler-derived preoperative MR volume is a powerful predictor of unexpected postoperative LV dysfunction. Prompt mitral valve repair may be beneficial for patients with high likelihood of successful repair and MR volume >/=80 mL.

Usefulness of left ventricular shape to predict the early recovery of left ventricular function after isolated aortic valve replacement for aortic valve stenosis.

Improvement in left ventricular (LV) systolic function after aortic valve replacement (AVR) has been observed in patients with aortic valve stenosis (AS). However, the factors that predict such recovery remain unclear. We sought to identify the predictive value of the LV spherical shape for LV functional recovery after "isolated" AVR in patients with severe AS and LV dysfunction. We examined 90 patients with severe AS and LV systolic dysfunction by echocardiography before and after AVR. Patients with known coronary artery disease, significant aortic or mitral regurgitation, and other cardiac surgery were excluded. LV end-diastolic and end-systolic volumes indexes and ejection fraction (EF) were measured by the Simpson method. LV mass index was calculated by the area-length method. LV end-diastolic and end-systolic sphericity were calculated as the ratio of the minor axis to the major axis of the left ventricle in apical 4-chamber view. The postoperative EF was significantly associated with preoperative EF, end-diastolic and end-systolic volumes indexes, LV mass index, and end-diastolic and end-systolic sphericity (all p <0.001). Multivariate analysis revealed that preoperative EF, end-systolic volume index, and end-diastolic sphericity were independent parameters predicting postoperative EF. The sensitivity and specificity in predicting normalization of EF (> or =50%) after AVR were 65% and 83% for end-diastolic sphericity <0.57 and 68% and 91% for end-systolic sphericity <0.47, respectively. In conclusion, LV spherical shape and dilatation predicted poor LV functional recovery after isolated AVR in severe AS.

Determination of regurgitant orifice area with the use of a new three-dimensional flow convergence geometric assumption in functional mitral regurgitation.

Geometry of the proximal isovelocity surface area (PISA) in functional mitral regurgitation (MR) is elongated, leading to underestimation of the effective regurgitant orifice (ERO) area. This underestimation could be corrected by a new hemiellipsoidal method. Thirty patients with functional MR were examined by real-time 3-dimensional (D) echocardiography. Two ERO areas were calculated from 3D measurements: ERO area by the hemispheric method and that by the new hemiellipsoidal method with our customized program. Each ERO area was compared with that by the 2D quantitative Doppler method. Color 3D images showed an elongated PISA geometry including 2 geometric types ("mountain" or "valley") in all patients with functional MR. Our hemiellipsoidal method could be adapted for all geometric types of PISA and underestimated ERO area by only 26%, whereas the underestimation by the hemispheric PISA method was 49%. The underestimation by the hemispheric PISA method can be significantly corrected by our hemiellipsoidal method.

Preoperative and postoperative mitral valve prolapse and regurgitation in adult patients with secundum atrial septal defects.

BACKGROUND: Little attention is given to development of mitral regurgitation (MR) in adults with atrial septal defect (ASD). The aim of the study was to determine the associated factors of MR in ASD adults before surgical repair and the fate of moderate to severe MR after surgery. METHODS: We examined 71 consecutive patients with secundum ASD (47 +/- 16 years) who underwent surgical repair. Clinical and echocardiographic variables including size of left and right heart systems and severity of MR and tricuspid regurgitation (TR) were investigated before and early after surgery. RESULTS: Before ASD closure, 14 patients (20%) had moderate to severe MR and 25 patients (35%) showed mitral valve (MV) prolapse. The ASD patients with moderate to severe MR showed worse cardiovascular symptoms, increased occurrence of atrial fibrillation and MV prolapse, and greater left ventricular (LV) end-diastolic volume, left atrial area, and TR severity than those with none to mild MR (all P < 0.05). Among preoperative variables, TR severity, left atrial area, LV end-diastolic volume, and MV prolapse were associated with preoperative MR severity in all the patients (all P < 0.03). Isolated ASD closure (n=46) decreased MV prolapse (P=0.008). Preoperative moderate to severe MR decreased after ASD closure with and without MV surgery (n=9 and 5, respectively; both P < 0.05). CONCLUSIONS: Preoperative MR severity was associated with TR severity, dilated left heart chambers, and MV prolapse. MR decreased after ASD closure with and even without MV surgery.

Diagnostic value of left ventricular outflow area in patients with hypertrophic cardiomyopathy: a real-time three-dimensional echocardiographic study.

BACKGROUND: Earlier studies demonstrated the ability of real-time 3-dimensional (3D) echocardiography (3DE) to measure left ventricular outflow tract (LVOT) area (A(LVOT)) in patients with hypertrophic cardiomyopathy (HCM). However, its clinical value is unknown. OBJECTIVE: We sought to investigate the feasibility and accuracy of real-time 3DE-derived A(LVOT) to diagnose significant LVOT obstruction in a large number of patients with HCM. METHODS: A total of 162 patients with HCM had 3DE by using a volumetric system. The smallest A(LVOT) during systole was determined by moving a 2-dimensional plane in 3D space. The pressure gradient across LVOT was assessed by continuous wave Doppler method. Provocation was performed in patients without significant LVOT obstruction (pressure gradient across LVOT < 50 mm Hg) at rest. RESULTS: Twenty (12%) patients with poor image quality of 3DE were excluded; 16 (28%) patients with a volumetric system, but only 4 (4%) patients with commercial equipment (P < .001). In the remaining 142 patients, A(LVOT) inversely correlated with pressure gradient across LVOT both at rest (r = 0.82, P < .001) and after provocation (r = 0.60, P < .001). The value of A(LVOT) less than 0.85 cm(2) and less than 2.0 cm(2) predicted resting and provokable LVOT obstruction with sensitivity of 87% and 81%, and specificity of 77% and 90%, respectively. CONCLUSIONS: Real-time 3DE measurement of A(LVOT) was successful in diagnosing and quantifying LVOT obstruction at rest and after provocation in a large number of patients with HCM.