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Objectives. We report the clinical course and unique hemodynamic findings after placement of a supraannular mitral valve prosthesis. Background. Children with symptomatic mitral valve disease whose annulus is too small for the smallest prosthesis are difficult to manage. One option is valve replacement with a prosthesis positioned entirely within the left atrium (LA). Methods. We reviewed 17 patients (median age 10 months) with symptomatic mitral valve disease who underwent placement of a supraannular valve prosthesis between 1980 and 1994. Results. The actuarial survival rates were 88% at 1 month and 71%, 62% and 53% at 1, 2 and 10 years, respectively. Preoperative hemodynamic data (mean ± SD) compared with those after placement of the supraannular mitral prosthesis were as follows: “a” wave to left ventricular end-diastolic pressure gradient 17 ± 5 versus 4 ± 4 mm Hg (p = 0.003), mean LA pressure 25 ± 6 versus 20 ± 6 mm Hg (p = 0.07), “a” wave 30 ± 6 versus 19 ± 5 mm Hg (p = 0.006), “v” wave 28 ± 5 versus 30 ± 9 mm Hg (p = 0.31), mean pulmonary artery pressure 54 ± 19 versus 42 ± 15 mm Hg (p = 0.07) and left ventricular end-diastolic pressure 14 ± 5 versus 16 ± 4 mm Hg (p = 0.12). Conclusions. Supraannular mitral valve replacement provides relief of mitral stenosis or mitral regurgitation. However, LA to left ventricular early diastolic gradients with large atrial “v” waves contribute to elevated mean LA pressures in the absence of prosthetic valve obstruction or regurgitation. As a result of this unexpected finding, associated left heart obstructive lesions and pulmonary and left ventricular end-diastolic hypertension, the outlook remains poor. (J Am Coll Cardiol 1997;29:1089–94) © 1997 by the American College of Cardiology

Objectives. We investigated the effect of inhaled nitric oxide and infused acetylcholine in patients with pulmonary hypertension undergoing cardiac catheterization before cardiopulmonary transplantation.Background. The fate of patients under consideration for transplantation of the heart or lungs, or both is influenced by the evaluation of their pulmonary vascular reactivity.Methods. We evaluated 11 patients who were classified into two groups on the basis of mean left atrial pressure >15 mm Hg (group I, n = 6) or ≤15 mm Hg (group II, n = 5). All patients inhaled nitric oxide at 80 ppm. This was preceded by an infusion of 10−6mol/liter of acetylcholine in seven consecutive patients (n = 3 in group I, n = 4 in group II).Results. In group I, inhaled nitric oxide decreased pulmonary artery pressure from (mean ± SE) 71 ± 13 to 59 ± 10 mm Hg (p < 0.05), pulmonary vascular resistance from 14.9 ± 3.8 to 7.6 ± 1.7 Um2(p < 0.05) and intrapulmonary shunt fraction from 17.8 ± 3.6% to 12.7 ± 2.1% (p < 0.05). Left atrial pressure tended to increase from 27 ± 4 to 32 ± 5 mm Hg (p = 0.07). In group II pulmonary vascular resistance decreased in response to nitric oxide from 36.4 ± 9.0 to 31.1 ± 7.9 Um2(p < 0.05). Cardiac index, systemic pressure and resistance did not change in either group. Seven patients who received acetylcholine had no significant alteration in pulmonary hemodynamic variables.Conclusions. These preliminary observations suggest that nitric oxide is a potent pulmonary vasodilator with minimal systemic effects. It may be useful in discriminating patients needing combined heart and lung transplantation from those requiring exchange of the heart alone.

Objectives. The aim of this study was to assess the morphology of the coronary sinus, its drainage and associated cardiac malformations when there is either complete unroofing of the coronary sinus or atresia of its connection to the right atrium. Background. As more children with complex cardiac anomalies are accepted for primary surgical repair or palliation with cavopulmonary anastomoses, a knowledge of coronary sinus and systemic venous anomalies is important if coronary venous return is to be preserved and residual shunts avoided. Methods. Twenty-six heart-lung specimens without a coronary sinus draining to the right atrium were identified from the Leiden collection of congenital heart malformations. These were classified into specimens with an unroofed coronary sinus and those with atresia of the coronary sinus orifice. Attention was paid to the associated cardiac malformations. Results. In 14 (54%; confidence limits [CL] 35%, 73%) of 26 specimens, there was an unroofed coronary sinus, associated with persistence of the left superior caval vein. An inferoposterior location of an atrial septal defect was detected in 2 (14%; CL –4%, 33%) of 14. Atrial appendage anomalies were seen in 13 (93%; CL 79%, 106%) of 14 specimens, exemplified by both right and left isomerism. These were frequently associated with an atrioventricular septal defect (12 [86%; CL 67%, 104%] of 14). An atretic coronary sinus orifice was seen in 12 (46%; CL 27%, 65%) of 26. Atrial appendage anomalies (2 [17%; CL –4%, 38%] of 12) were rare in these cases. The drainage was then by way of a left superior caval vein or, in its absence, a coronary sinus to left atrial window. Ventricular hypoplasia was seen in both categories of coronary sinus abnormalities. Important ventricular hypoplasia was seen in 12 cases (46%; CL 27%, 65%). Conclusions. These findings emphasize the need to study coronary sinus drainage before procedures such as ligation or transcatheter coil embolization of a left superior caval vein, venous redirection or closure of a dorsal atrial septal defect are contemplated. These procedures might inadvertently lead to impairment of coronary venous return or persistence of an intracardiac shunt.

ObjectivesWe investigated the effects of different CO2tensions on oxygenation, pulmonary blood flow (Qp), cerebral blood flow, and systemic blood flow (Qs) after the bidirectional superior cavopulmonary anastomosis (BCPA).BackgroundHypoxemia refractory to management of a high pulmonary vascular resistance index (PVRI) may complicate recovery from the BCPA.MethodsAfter BCPA, CO2was added to the inspired gas of mechanically ventilated patients. The Qp, Qs, PVRI, and systemic vascular resistance index (SVRI) were calculated from oxygen consumption, intravascular pressures, and oxygen saturations. Cerebral blood flow was estimated by near infrared spectroscopy and transcranial Doppler.ResultsIn nine patients (median age 7.1, range 2 to 23 months), arterial oxygen tension increased significantly (p < 0.005) from 36 ± 6 mm Hg to 44 ± 6 to 50 ± 7 mm Hg at arterial carbon dioxide tensions (PaCO2) of 35, 45, and 55 mm Hg, respectively and decreased to 40 ± 8 mm Hg at PaCO240 mm Hg. At a PaCO2of 55 and 45 compared with 35 mm Hg, Qp, cerebral blood flow, and Qs increased significantly, PVRI, Qp/Qs, and the ratio of Qp to inferior vena caval blood flow were unchanged, but SVRI decreased.ConclusionsWe have demonstrated that after the BCPA, systemic oxygenation, Qp, Qs, and cerebral blood flow increased and SVRI decreased at CO2tensions of 45 and 55 mm Hg compared with 35 mm Hg. We suggest that hypoxemia after the BCPA is ameliorated by a higher PaCO2and that low PaCO2or alkalosis may be detrimental. Hypercarbic management strategies may allow earlier progression to the BCPA, which may contribute to reducing the interval morbidity in patients with a functional single ventricle.

Objectives. We reviewed the factors contributing to or causing death before surgery in neonates with d-transposition of the great arteries (TGA) despite anatomy suitable for the arterial switch operation (ASO) to develop strategies to minimize preoperative attrition.Background. Currently the ASO for neonates with TGA carries a low operative mortality. However, there is a paucity of information regarding the patients who die before the ASO. Strategies to ensure survival to operation are of importance to improve overall outcome.Methods. We reviewed all neonates with TGA and patent forearm ovale (PFO) ≤2 mm, a birthweight

This study was undertaken to investigate the long-term outcome of balloon angioplasty for recurrent coarctation of the aorta in a large series of patients.Balloon angioplasty has become the standard treatment for residual or recurrent aortic coarctation. Despite the widespread use of this treatment modality, there are few data outlining the long-term outcome of a large patient cohort.Clinical, echocardiographic, hemodynamic and angiographic data on 90 consecutive patients who underwent balloon angioplasty between January 1984 and January 1996 were reviewed.Mean systolic pressure gradients were reduced from 31 +/- 21 to 8 +/- 9 mm Hg after dilation (p = 0.0001). The mean diameter of the stenotic site, measured in the frontal and lateral views, increased by 38% and 35%, respectively (p = 0.001). Neurologic events occurred in two patients, with one death. An aortic tear occurred in one patient, requiring surgical intervention. Optimal results were defined as a postprocedure gradient20 mm Hg and were obtained acutely in 88% of patients. At long-term follow-up (12 years), 53 (72%) of 74 patients with an early optimal result remained free from reintervention. Transverse arch hypoplasia, defined as an arch dimension2 SD below the mean for age, was the primary predictor of the need for reintervention.Although the majority of patients undergoing percutaneous balloon angioplasty for recoarctation of the aorta will achieve long-term benefit, the need for further surgical intervention in those with transverse arch hypoplasia remains high.

Pulmonary arterial hypertension during childhood is a heterogeneous disease that appears to have a genetic basis, and mutations involving bone morphogenetic protein receptor 2, activin receptor-like kinase 1, endoglin, and transforming growth factor beta receptors have been documented ([1–4][1]).

In 1998, a clinical classification of pulmonary hypertension (PH) was established, categorizing PH into groups which share similar pathological and hemodynamic characteristics and therapeutic approaches. During the 5th World Symposium held in Nice, France, in 2013, the consensus was reached to maintain the general scheme of previous clinical classifications. However, modifications and updates especially for Group 1 patients (pulmonary arterial hypertension [PAH]) were proposed. The main change was to withdraw persistent pulmonary hypertension of the newborn (YPPH) from Group 1 because this entity carries more differences than similarities with other PAH subgroups. In the current classification, PPHN is now designated number 1". Pulmonary hypertension associated with chronic hemolytic anemia has been moved from Group 1 PAH to Group 5, unclear/multifactorial mechanism. In addition, it was decided to add specific items related to pediatric pulmonary hypertension in order to create a comprehensive, common classification for both adults and children. Therefore, congenital or acquired left-heart inflow/outflow obstructive lesions and congenital cardiomyopathies have been added to Group 2, and segmental pulmonary hypertension has been added to Group 5. Last, there were no changes for Groups 2, 3, and 4. (J Am Coll Cardiol 2013;62:D34-41) a 2013 by the American College of Cardiology Foundation.

OBJECTIVESWe compared the ability of inhaled nitric oxide (NO), oxygen (O2) and nitric oxide in oxygen (NO+O2) to identify reactive pulmonary vasculature in pulmonary hypertensive patients during acute vasodilator testing at cardiac catheterization.BACKGROUNDIn patients with pulmonary hypertension, decisions regarding suitability for corrective surgery, transplantation and assessment of long-term prognosis are based on results obtained during acute pulmonary vasodilator testing.METHODSIn group 1, 46 patients had hemodynamic measurements in room air (RA), 100% O2, return to RA and NO (80 parts per million [ppm] in RA). In group 2, 25 additional patients were studied in RA, 100% O2and 80 ppm NO in oxygen (NO+O2).RESULTSIn group 1, O2decreased pulmonary vascular resistance (PVR) (mean ± SEM) from 17.2 ± 2.1 U·m2to 11.1 ± 1.5 U·m2(p < 0.05). Nitric oxide caused a comparable decrease from 17.8 ± 2.2 U·m2to 11.7 ± 1.7 U·m2(p < 0.05). In group 2, PVR decreased from 20.1 ± 2.6 U·m2to 14.3 ± 1.9 U·m2in O2(p < 0.05) and further to 10.5 ± 1.7 U·m2in NO+O2(p < 0.05). A response of 20% or more reduction in PVR was seen in 22/25 patients with NO+O2compared with 16/25 in O2alone (p = 0.01).CONCLUSIONSInhaled NO and O2produced a similar degree of selective pulmonary vasodilation. Our data suggest that combination testing with NO+O2provides additional pulmonary vasodilation in patients with a reactive pulmonary vascular bed in a selective, safe and expeditious fashion during cardiac catheterization. The combination of NO+O2identifies patients with significant pulmonary vasoreactivity who might not be recognized if O2or NO were used separately.

Portopulmonary hypertension (PPH) and hepatopulmonary syndrome (HPS) are risk factors for adverse outcomes after liver transplantation in adults. We sought to determine the incidence of PPH and HPS in children referred for liver transplantation. We reviewed the clinical findings, electrocardiograms