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It emerges from the posterior and rightward aspect of intervention because they are genetically separate from more the main pulmonary artery 10mg triamcinolone free shipping treatment junctional tachycardia. The left pulmonary artery arises from the of underdevelopment of left heart structures cheap triamcinolone online american express treatment 1st degree av block. It often appears to be slightly smaller than The mitral valve may be either stenotic or atretic generic triamcinolone 15mg line treatment narcolepsy, as may the right pulmonary artery purchase triamcinolone treatment xanax withdrawal. Occasionally, the foramen • aortic atresia and mitral stenosis ovale is severely restrictive. There is also generally increased muscu- Boston, 35% had aortic atresia/mitral atresia and 20% had larization of the walls of the pulmonary veins. Aortic atresia tends to be associated with a more severe degree of hypoplasia of the AssociAted cArdiAc AnomAlies ascending aorta than does aortic stenosis. A coarctation coworkers16 reported the presence of coronary artery steno- shelf is present opposite the junction of the ductus with the ses in more than 50% of patients in the subgroup with aortic proximal descending aorta in at least 80% of patients. By con- a direct extension of the main pulmonary artery, which is trast, coronary artery anomalies were rarely seen in patients even larger (11–15 mm in diameter, and occasionally even with aortic atresia and mitral atresia. Thus, exposure of the neonate to at the Children’s Hospital of Philadelphia, Natowicz et al. Overall, 28% had genetic disorders, major inadequate systemic fow will be further exacerbated by even extracardiac anomalies, or both. Complete closure of the ductus ties included Turner syndrome and trisomies 18, 13, and 21. Excessive pulmonary blood fow Anomalies not associated with chromosomal defects included can also result from inappropriate balloon dilation of the diaphragmatic hernia, hypospadias, and omphalocele. The mildly to authors found that, overall, 29% of the 41 autopsies revealed moderately restrictive atrial septum performs the important either a major or minor central nervous system abnormality. The absence of dysmorphic physical features did not preclude overt or subtle central nervous system malforma- Prenatal diagnosis is very common today so that the new- tions. Right ventricular out- ter specializing in neonatal and infant cardiac surgery while put is directed across the ductus, where it can pass antegrade receiving a prostaglandin E1 infusion; supplemental oxygen down the descending aorta or retrograde around the aortic should be avoided. Many transport teams prefer to electively arch to the head vessels and the ascending aorta, which intubate such children because of the risk of apnea induced functions as a single coronary artery. Facilities must be available to ventilate Children’s National Medical Center have documented that the child with room air when the child is intubated. Although when out of hospital the child patent, the child’s continuing viability will be dependent on is free of the risk of exposure to supplemental oxygen (which 448 Comprehensive Surgical Management of Congenital Heart Disease, Second Edition in the past often resulted in deterioration while the child was Prenatal diagnosis allows for expeditious transfer of the child in the hospital), there is a risk that the ductus will close pre- to the tertiary care facility immediately after birth. Preferably cipitously or when the child is at a location geographically the obstetrical care should be undertaken in a facility imme- remote from medical attention. Under such circumstances diately adjacent to the pediatric center33 or even within the the child is likely to develop serious metabolic acidosis and pediatric center itself if immediate care will be needed, such may be in a state of profound shock with cardiovascular col- as balloon dilation of a severely restrictive atrial septum. The physical fndings of a slightly cyanotic neo- secondary to this acidotic insult, resulting in seizures, renal nate in respiratory distress, with a variable degree of general failure, hepatic failure, and depressed ventricular function. Likewise, the appear- The reversibility of these various organ defcits will depend ance on the chest X-ray of a slightly enlarged heart with con- on both the severity of the acidosis and its duration. Echocardiography the point where the child is profoundly cyanotic from the will generally provide excellent defnition of the relevant fea- moment of birth. This situation cannot be palliated medi- of the neonate usually guarantees excellent defnition of this cally but requires urgent intervention in the catheterization area, which may not be as clearly seen in an older infant or laboratory to open the atrial septum. It is usually possible to defne whether a shelf or coarc- restrictive or intact atrial septum is diagnosed prenatally and tation is present opposite the insertion of the ductus; this is is opened either by an in utero intervention or immediately encountered in 80% of neonates with this anomaly. In many cases the diagnosis however, may infuence the surgeon to reconstruct the neo- can be made confdently by 16–18 weeks of gestation and aorta as a tube graft with end to side reimplantation of the with transvaginal ultrasound even frst trimester diagnosis is tiny ascending aorta rather than the traditional cuff repair feasible. In one case necessary to pass a catheter either through or close to the report Maxwell, Allan, and Tynan described identifcation ductus. The osmotic load of angiographic dye was a study and had become hypoplastic and densely echogenic. In addition, there were the general tion invites prenatal intervention, either in the form of echo- stresses inherent in any cardiac catheterization procedure cardiographically guided balloon dilation of the aortic valve, (heat loss, blood loss, and the catecholamine response to the as frst reported by Maxwell and associates,32 or by surgical stress of the procedure). The topic is cov- and are familiar with the most current results; the maternal ered in detail in Chapter 22, Left Ventricular Outfow Tract fetal medicine and obstetrical team may not be in a posi- Obstruction: Aortic Valve Stenosis, Subaortic Stenosis, and tion to answer more detailed inquiries posed by the parents. In summary, the calculator Hypoplastic Left Heart Syndrome 449 developed by the Congenital Heart Surgeons’ Society (avail- brain injury, although whether this applies in neonates is not able at www. Nevertheless, it would seem wise to avoid very high mining whether an individual child should be managed with levels of blood glucose. Clear lines of communication are essential so to all organ systems other than the cardiorespiratory system that discussion can ensue regarding such issues as whether itself. Failure to achieve this goal will defnitely jeopardize to pursue a univentricular or biventricular approach (balloon the outcome of surgery. Early infusion of prostaglandin is including the child’s parents, is that only general supportive currently practiced at most referral centers before defnitive measures should be given. If the important psychologic advantage for most prospective par- child continues to have poorly palpable pulses or if the blood ents, who can prepare themselves logistically, intellectually, pressure measured by an umbilical arterial line is low, a care- and emotionally for the hurdles that may lie ahead for them ful check should be made to ensure that the prostaglandin is and that can be explained in detail several months before being delivered into the bloodstream. On the other hand, for some parents the worry venous line with excellent blood return is the safest venous access at this stage. If the team is sure that the prostaglandin induced by fetal diagnosis can be counterproductive and may is being delivered at an adequate dose (initially 0. When the min), consideration should be given to supporting the child diagnosis is made after delivery, particularly if the child has with a dopamine infusion, beginning at a dose of 5 µg/kg/ been at home for some time and recognition of the child’s min up to 20 µg/kg/min. For many families, parent support groups are of great child receiving prostaglandin who may become apneic in the help, particularly after the child has left the hospital and the confned area of a transport vehicle, where intubation will parents are faced with the prospect of future hospital visits, be hazardous. Elective intubation before transport should be cardiac catheterizations, and surgery. The general principles of are attending school and leading normal lives after comple- neonatal resuscitation and transport, such as maintenance of tion of surgical treatment is a great emotional boost to parents adequate body temperature and blood glucose levels, should who may be anticipating caring for a child who is chronically be carefully adhered to. Likewise, there is evidence Details are described in Chapter 4, Pediatric Cardiac that hyperglycemia in mature animals exacerbates ischemic Intensive Care. If the septum is opened inappropriately or excessively, torrential pulmonary blood fow can result lead- Prenatal ing to a worsening metabolic acidosis as systemic blood fow Remarkable advances in obstetrical ultrasound imaging and becomes inadequate. Urgent stage 1 Norwood surgery may the development of interventional catheter hardware and be necessary. However, whether the risk/ben- usual practice at Children’s National Medical Center to eft ratio justifes this approach is still unclear. For example, undertake dilation of the aortic valve under these circum- the team at Guy’s Hospital London led by Tynan who pio- stances because of concern that the valve might become neered the approach in the early 1990s32 eventually stopped regurgitant. The technique has subsequently that combine surgical and interventional catheter proce- been promoted by the team at Children’s Hospital Boston dures. The catheter procedures include stenting of the ductus who believe that improved fetal echocardiography allowing and balloon dilation of the atrial septum. Pulmonary blood earlier diagnosis and intervention is more likely to achieve fow is restricted by application of bilateral pulmonary artery success, that is, avoidance of the single ventricle track.
Washing Southern blot membranes at different temperatures results in different stringencies purchase triamcinolone with a visa treatment quad tendonitis. Lower-temperature washes can reveal sequences on the membrane that are similar order triamcinolone online pills medications ending in zole, but not identical purchase 40mg triamcinolone with amex treatment 8th march, to the sequence of the probe order cheap triamcinolone treatment tmj. The actual temperature at which washes are performed will depend on the length of the probe and the likelihood of it ﬁnding an exactly matching sequence on the membrane will give positive signals on the X-ray ﬁlm. This approach is immensely useful when you do not precisely know the sequence of the gene you wish to identify. Such techniques have been assigned names reﬂecting the points of a compass rather than a description. The proteins are transferred from the gel onto a membrane in much the same way as described above for Southern blotting. The speciﬁc interaction between the antibody and its antigen occurs on the membrane, and the position of the bound antibody is detected. Initially radio-labelled antibodies were used, but these have been largely superseded by antibody ‘sandwiches’. The sandwiches work through the binding of one unlabelled antibody (the primary antibody) to the antigen on the membrane. A second, labelled, antibody (the secondary antibody) is then used to detect the presence of the ﬁrst antibody. This has several advantages; ﬁrstly, the multivalent nature of antibody binding means that a substantial increase in sensitivity is achieved, and secondly, a single secondary antibody can be used to detect a number of different primary antibodies. A secondary antibody raised, say, in the rabbit against mouse immunoglobulins will be capable of interacting with a number of different mouse derived primary antibodies (Figure 2. The isolation of nuclear material from cells is a relatively straightforward process. Non-speciﬁc protein binding sites are blocked on the membrane – using solubilized milk powder – before the primary antibody is added. A labelled secondary antibody is then added to detect the location of the primary antibody. The secondary antibody is often labelled with an enzyme whose activity, in the presence of appropriate substrates, results in either a colour change on the membrane or the emission of light that can be detected using X-ray ﬁlm the cell wall will result in the nuclear material spilling out from the broken cells. The method used for the lysis procedure depends upon the nature of the host cell itself. For instance, bacterial cells are often treated with the enzyme lysozyme to weaken their cell wall before being lysed with detergents. Yeast cells, on the other hand, are treated with zymolase to disrupt the integrity of their cell wall before lysis proceeds – often by grinding the cells using glass beads to break them open. Chloroform is often used in conjunction with phenol (as a phenol/chloroform solution) since it is also a protein denaturant, but it also stabilizes the rather unstable boundary between the aqueous phase and a pure phenol layer. Insufﬁcient cell lysis will result in low plasmid yields, while cells that have been lysed too much suffer similar problems. This method, termed the alkaline lysis procedure, takes advantage of the fact that at alkali pH (between 12. Under these conditions, other contaminants will not bind to the matrix and can be washed away. Escherichia coli, named for the German physician Theodor Escherich (1857–1911), is a gram-negative, rod shaped bacterium propelled by long, rapidly rotating ﬂagella (Figure 3. It is part of the normal ﬂora of the human mouth and gut, helping to protect the intestinal tract from bacterial infection, aiding digestion and producing small amounts of vitamins B12 and K. The bacterium, which is also found in soil and water, is widely used in laboratory research and is probably the most thoroughly studied life form. A cut-away model of the bacterium showing some of the cellular layers and components, and E. As far back as the mid-1940s it was known that bacterial cells were able to exchange genetic material with each other in a semi- sexual manner. The experiments of Lederberg and Tatum clearly demonstrated the transfer of genetic information through bacterial conjugation (Lederberg and Tatum, 1946). The ability to perform this transfer is conferred by a set of genes called F (for fertility). When conjugation occurs, the F genes start travelling across the pilus, dragging the rest of the genome behind them. The bacterial genome can be measured, in minutes, from the origin of transfer with the amount of time it takes for a particular gene to be transferred from one bacterium to another indicating how far it is from the origin of replication. However, the size of the F episome precludes easy analysis and manipulation and its gene transfer properties can make it unstable. Most commonly used vectors are based either upon plasmids or bacteriophage lambda (λ). In general, vectors can be thought of as a series of discrete modules that provide requirements essential for efﬁcient molecular cloning. A huge array of different types of vector is available today, with many being highly specialized and designed to perform a speciﬁc function. In this chapter, I will discuss some of the general points of vector design, but will concentrate on vectors that are commonly used in cloning experiments (Table 3. Plasmids are widely distributed throughout prokaryotes and range in size from approximately 1500 bp to over 300 kbp. The replication of the plasmid is often coupled to that of the host cell in which it is maintained, with plasmid replication occurring at the same time as the host genome is replicated. Plasmids are often described as being either relaxed or stringent on the basis of the number of copies of the plasmid that are maintained within the cell. At least part of the basis of this difference is the different mechanisms employed by plasmids in order to replicate themselves. In general, relaxed plasmids replicate using host derived proteins, while stringent plasmids encode protein factors that are necessary for their own replication. Other gene names reﬂect biological function, for example, Hbb for the haemoglobin β-chain, and Adh for alcohol dehydrogenase enzymatic activity. Drosophila geneticists have brought the most whimsical approach gene naming – with names like fushi tarazu (ftz, from the Japanese words meaning ‘segment deﬁcient’), spatzle¨ (spz, a type of German noodle), dunce (dnc), forkhead (fkh), hedgehog (hh)andether-a-go-go (eag). The assignment of chromosomal locations for genes of unknown function developed soon after the establishment of successful metaphase spreads, chromosome banding methodologies, somatic cell hybrids, isozyme separation and the ability to associate genes and phenotypes with a particular site on the chromosome. It has also become common to use the same name for the gene as for the enzyme or other protein that it encodes. Often, the gene name is italicized whereas the gene product is not to distinguish between the two. This can, however, be a source of confusion, since there is not necessarily a one-to-one relationship between the two entities. Additionally, nomenclature used for one organism or species may be different in another. Traditionally, recombinant plasmids tend to bear the initials of their creator(s) followed by a number that may indicate the numerical order in which the plasmids were produced, or perhaps has some deeper meaning.
Additionally buy triamcinolone now pretreatment, delivery at order triamcinolone 10 mg symptoms stomach cancer, or transport to a facility equipped to handle these newborns can be planned generic triamcinolone 40mg with visa medicine doctor, along with appropriate postnatal management purchase triamcinolone american express symptoms white tongue. Cardiac Catheterization/Angiography These days, cardiac catheterization is not needed for diagnosis. In a previous era, the “laid-back” angiogram was performed to evaluate the coronary arteries (45). This can also be performed if the patient undergoes a preoperative catheterization for other purposes, or the coronary artery pattern cannot be readily discerned by echocardiography (although rare), and the information is felt mandatory by the surgical team (not usually necessary). This is performed by angulating the frontal camera in an extreme caudal projection. A balloon occlusion angiogram in the ascending aorta with a catheter that has side holes proximal to the balloon, for example, a Berman angiographic catheter (Arrow International, Inc. This same angulation may be used from the femoral arterial approach after an arterial switch operation to examine the coronary arteries if needed. Selective coronary angiography can be safely performed even in very small infants. On the right, a dilated neo-aortic root (arrow) is seen, a common long-term finding in these patients. Extreme caudal angulation is placed on the anterior–posterior projection (∼45 degrees) and a balloon occlusion angiogram is performed in the aorta. Note that this newborn has a single coronary artery ostium (arrow), giving rise to the right coronary artery (single asterisk), left anterior descending coronary artery (double asterisks) and circumflex coronary artery (triple asterisks). Natural History and Management Natural History and Immediate Outcomes Untreated, transposition of the great arteries is a fatal disease. A unique study from a large database between 1957 and 1964 in the state of California performed by Liebman et al. The advent of the Blalock Hanlon procedure (surgical atrial septal defect creation) (47) and various partial venous redirection procedures (e. In addition to these procedures, the less invasive balloon atrial septostomy procedure (2) dramatically changed the natural history and early survival of these infants (50,51). A: The operation is performed utilizing hypothermic cardiopulmonary bypass with cannulation of the superior and inferior vena cavae. The aorta is cross-clamped and the myocardium is protected with intermittent doses of cold cardioplegic solution. B: An atrial septal flap is created by incising the septum on its anterior, inferior, and superior aspects. Note that there is atrioventricular concordance so that the mitral valve is left sided and the tricuspid valve right sided. C: The flap is now turned down into the left atrium and is sutured around the anterior, inferior, and superior margins of the orifices of the pulmonary veins (blue suture line). This flap isolates the pulmonary veins from the left atrium and forms the roof of the pulmonary venous chamber and the floor of the systemic venous chamber. The posterior right atrial wall is then used as a flap to construct the anterior aspect of the vena caval pathway to the mitral valve. It is sutured around the orifices of the superior and inferior vena cavae and brought to the edge of the septum between the tricuspid and mitral valves (red dotted line). The anterior right atrial wall is used as a flap to construct the pulmonary venous pathway from the left atriotomy to the tricuspid valve (orange dotted line). Pulmonary venous return is directed to the aorta via the tricuspid valve and right ventricle (red arrow) and systemic venous return is directed to the pulmonary artery via the mitral valve and left ventricle (blue arrows) leading to a physiologic correction at the atrial level. This leaves the morphologic right ventricle as the systemic ventricle and the tricuspid valve as the systemic atrioventricular valve. Results of the Senning and Mustard procedures were reproducible with early survival approaching generally 90% in experienced centers and patients achieved a normal oxygen saturation. Data from the Congenital Heart Surgeons Study showed the early mortality from the Senning and Mustard procedures to be 11% and 0%, respectively (54). Further follow-up showed survival for these atrial redirection procedures was 90% and 85% at 1 month and 5 years, respectively, with survival in the Mustard group being better (55). A: The operation is performed utilizing hypothermic cardiopulmonary bypass with cannulation of the superior and inferior vena cavae. The aorta is cross-clamped and the myocardium is protected with intermittent doses of cold cardioplegic solution. B: The atrial septum and majority of the limbus are resected to create a large atrial septal defect that extends to the superior and inferior vena cava. C: The large interatrial communication has been created exposing the pulmonary veins. Note that there is atrioventricular concordance so that the mitral valve is left sided and the tricuspid valve right sided. D: An intra-atrial baffle (shaded), usually of pericardium, is constructed to direct the vena caval flow to the mitral valve. The suture line is begun anterior to the left-sided pulmonary veins between the veins and the orifice of the left atrial appendage. E: The baffle suture line is then brought inferior to the pulmonary veins and laterally to the right atrial–inferior vena caval junction, anteriorly around the inferior vena caval orifice, and to the retained anterior portion of the atrial septum. In a similar fashion, the superior edge of the baffle is brought superior to the pulmonary veins and laterally to the right atrial–superior vena caval junction, anteriorly around the superior vena caval orifice, and onto the anterior portion of the atrial septum to complete the suture line. Pulmonary venous return is directed around the baffle to the aorta via the tricuspid valve and right ventricle (red arrow), and systemic venous return is directed under the baffle to the pulmonary artery via the mitral valve and left ventricle (blue arrows) leading to a physiologic correction at the atrial level. This leaves the morphologic right ventricle as the systemic ventricle and the tricuspid valve as the systemic atrioventricular valve. A: External anatomy with the aorta arising from the right ventricle (situs solitus with atrioventricular concordance and ventriculoarterial discordance). B: The operation is performed utilizing hypothermic cardiopulmonary bypass with aortic and bicaval cannulation. The aorta is cross-clamped and the myocardium is protected with intermittent doses of cold cardioplegic solution. If a ventricular septal defect is present it can be repaired at this time via a right atriotomy incision. The aorta is then transected several millimeters above the aortic valve commissures. Note that the ductus arteriosus has been ligated and divided to allow for later mobilization of the pulmonary artery bifurcation anterior to the aorta (Lecompte maneuver). D: The coronary arteries are harvested on buttons of aortic sinus tissue and mobilized to allow for translocation to the pulmonary artery (neo-aortic root). Medially based trap-door incisions are created in the pulmonary root at the translocation sites. These trap-door incisions prevent rotation of the coronaries and allow for the coronaries to sit in a natural orientation when translocated. E, F: The coronary buttons are translocated to the pulmonary root and sutured in place. G: The pulmonary artery bifurcation is brought anterior to the ascending aorta (Lecompte maneuver) and an end-to-end anastomosis is performed between the neo-aortic root and the ascending aorta. The Lecompte maneuver allows for a direct pulmonary artery anastomosis and avoids the need for prosthetic conduit reconstruction of the pulmonary outflow tract (61).
We prefer the definition of a ventricle as an endocardial-lined chamber within the ventricular mass buy triamcinolone with american express medications not to be taken with grapefruit, regardless of the ventricular components that are present or absent order 4mg triamcinolone with amex symptoms bladder cancer. Patients with any type of single functional ventricle are amenable to a modified Fontan type of surgical repair cheap triamcinolone 40mg with mastercard treatment 1 degree burn. We will use the term “single functional ventricle” to help avoid any conceptual confusion that terms like “univentricular heart” and “single ventricle” might cause purchase triamcinolone 4mg with amex medicine jar. In this anomaly, the rudimentary right ventricle is located anterior and superior to the dominant left ventricular chamber. In short axis, the plane of the ventricular septum will be angled rather than perpendicular to the diaphragmatic ventricular wall and it will be displaced superiorly. In this condition, the hypoplastic left ventricular chamber will be located along the inferior (diaphragmatic) aspect of the heart, usually to the right or left of midline. Rarely, the morphology of neither the dominant chamber nor the hypoplastic chamber can be determined with certainty. Such cases are typically categorized as single functional ventricle of undifferentiated or indeterminate type. When one of the valves straddles into the hypoplastic right ventricle, it will have a hybrid mitral–tricuspid morphology. These hearts must be distinguished from those in which there is an imperforate valve orifice (Figs. Note the associated effect of increasing atrial and ventricular septal malalignment produced by increasing annular override. A: The series of anomalies that link the different types of double-inlet ventricles with the normal heart when there has been right-hand (D) embryonic ventricular looping. B: The comparable series of double-inlet ventricles when there has been left-hand (L) embryonic ventricular looping. The valve that straddles is almost always on the same side as the hypoplastic outlet chamber. For the rest of the patients in their series, 23% were found to have transposition of the great arteries of the d-loop variety (i. Other common combinations described in the literature include the pattern seen with univentricular heart of right ventricular morphology. In the univentricular heart of indeterminate morphology, as there is truly a single ventricle there can only be a double outlet or a single outlet. This can occur with either concordant or discordant ventriculoarterial connections. When subpulmonary obstruction occurs, it typically is present within the ventricle of left ventricular morphology and is due to posterior deviation of the infundibular septum. In rare cases, there can be severe stenosis at the level of the pulmonary valve due to malformed leaflets or annulus hypoplasia. The communication often can be restrictive, occurring in 47% of cases in the series published by Bevilacqua et al. Even when it is unrestrictive at birth, the defect will often become restrictive over time. However, when transposition of the great arteries was present without pulmonary stenosis, a restrictive outlet foramen was significantly more common. In each of these clinical forms, other common associations include subaortic obstruction, pulmonary outflow tract obstruction, and conduction abnormalities (6). It also can be secondary to severe ventricular hypertrophy of muscle bundles within the hypoplastic right P. When subpulmonary obstruction occurs within the left ventricular chamber, it is most frequently due to posterior deviation of the infundibular septum. The nonbranching bundle then descends toward the crest of the trabecular septum along the left ventricular aspect of the ventricular septum and branches beneath the septal crest. The nonbranching bundle therefore must run a more extensive course anterior to the posterior semilunar valve annulus to reach the trabecular septum (Fig. In all instances, it is located on the left ventricular aspect of the trabecular septum and descends down the right rim of the ventricular septum, branching below the septal crest. The conduction tissue enters the trabecular septum from an anterolateral node (7). Subpulmonary stenosis is present because of posterior deviation of the conus septum. The aorta (Ao) overrides the ventricular septum producing a tunnel-like fibromuscular stenosis. There also is significant variation in the size of the morphologic right ventricular cavity. Subaortic obstruction is an important associated lesion that must be assessed when considering a modified P. Significant ventricular hypertrophy substantially increases operative risk for Fontan operation because of associated ventricular diastolic abnormalities and elevated left ventricular end-diastolic pressure. Double-Inlet Right Ventricle Double-inlet right ventricle was found in only two patients, or 5%, of the series reviewed by Van Praagh et al. The ventricular myocardium had coarse, straight trabeculations consistent with right ventricular morphology. However, investigators in more recent reviews (6) noted the presence of a hypoplastic rudimentary left ventricular chamber that usually can be recognized by careful angiographic or echocardiographic analysis. This ventricular relationship is consistent with an embryologic d-ventricular loop. There is considerable variability in the size of the hypoplastic left ventricular chamber, but most often it is very hypoplastic. Frequently, pulmonary stenosis or atresia is present with infundibular and pulmonary annular hypoplasia. The pathologic descriptions of these forms of double-inlet ventricle often share similar features. A: Pathologic features of mixed or indeterminate ventricular morphology with coarse trabeculated myocardium on the right and smooth-walled myocardium on the left (arrows) with emphasis in insert. B: Pathologic specimen demonstrating double-inlet ventricle (V) of indeterminate morphology. Double-Inlet Ventricle of Mixed Morphology Double-inlet ventricle of mixed morphology is a rare form of univentricular connection and occurred in only 5% of the series reported by Van Praagh et al. Also called a common ventricle, it was designated by Van Praagh as the C type of single ventricle with absence of the ventricular septum or undivided ventricles with a rudimentary septum. A small apical ridge of ventricular septum may often separate the right and left ventricular zones of the heart. Relationships of the ventricular zones are usually consistent with normal ventricular locations or d- ventricular looping.
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