Sequential Segmental Approach Research Articles

Contemporary sequential segmental approach to congenital heart disease using four-dimensional magnetic resonance imaging with ferumoxytol: an illustrated editorial.

The ferumoxytol-enhanced 4D MR angiography with MUSIC (Multiphase Steady State Imaging with Contrast) technique provides a single data set that captures dynamic cardiovascular anatomy and ventricular function at the same time. Homogeneous opacification of all cardiovascular structures within the imaging volume allows full sequential segmental approach to the congenital heart diseases without any blind spots. The complex systemic and pulmonary venous anatomy is particularly well captured in the MUSIC. Cinematographic display of multiplanar sectional and 3D volume images is helpful in the morphological identification of the cardiac chambers, the assessment of the dynamic nature of the ventricular outflow tracts, and the assessment of the coronary arterial origins and courses.

Sequential Segmental Approach to Congenital Heart Disease

The sequential segmental approach is essential for better understanding of cardiac anatomy in normal and malformed hearts. It is based on a detailed analysis of the three main cardiac segments, namely atria, ventricles, and great vessels, and the two connecting segments, namely atrioventricular and ventriculoarterial connections. Each segment is systematically defined based purely on its morphological characteristics. In most cases, echocardiography is sufficient, but some cases necessitate the use of other imaging modalities. Systematic identification of different segments, connections, and their abnormalities helps in making an accurate diagnosis of congenital heart disease (CHD). This review provides a brief description of the sequential segmental approach for detecting CHD on echocardiography.

AIUM Practice Parameter for the Performance of Fetal Echocardiography.

AIUM Practice Parameter for the Performance of Fetal Echocardiography.

Sequential segmental analysis of the crocodilian heart.

Differences between hearts of crocodilians and those of mammals and birds are only partly understood because there is no standardised approach and terminology for describing cardiac structure. Whereas most reptiles have an undivided ventricle, crocodilians have a fully septated ventricle. Their hearts, therefore, are more readily comparable with the hearts of mammals and birds. Here, we describe the heart of a crocodile (Crocodylus noliticus). We use the versatile sequential segmental approach to analysis, juxtaposing several key views of the crocodilian heart to the comparable views of human hearts. In crocodiles, the atrial and ventricular septums are complete but, unlike in placental mammals, the atrial septum is without an oval fossa. The myocardial component of the crocodilian ventricular septum dominates, but the membranous septum likely makes up a greater proportion than in any mammal. In the crocodile, the aortic trunk takes its origin from the left ventricle and is not wedged between the atrioventricular junctions. Consequently, there is a common atrioventricular junction, albeit with separate right and left atrioventricular valvar orifices. As in mammals, nonetheless, the crocodilian left atrioventricular valvar orifice is cranial to the right atrioventricular valvar orifice. By applying a method of analysis and terminology usually restricted to the human heart, we build from the considerable existing literature to show neglected and overlooked shared features, such as the offset between the left and right atrioventricular valvar orifices. Such commonalities are surprising given the substantial evolutionary divergence of the archosaur and synapsid lineages, and likely reflect evolutionarily shared morphogenetic programmes.

Heterotaxy Syndrome: An Embryologic Study

Heterotaxy is a clinically and genetically heterogeneous disorder with two main settings - left or right isomerism. Important diagnostic pointers like viscerocardiac heterotaxy, complex cardiac malformations, anomalies of the inferior vena cava may provide the prenatal diagnosis. Special signs of heterotaxy were sonographically detected in 5 human foetuses. Following the termination of the pregnancy a detailed examination according to the guidelines of the sequential segmental approach was performed. The main findings include multiple failures in the formation of the heart resulting from the dysfunctional viscerocardiac arrangement. The discontinuity and malposition of the inferior vena cava as well as the duplication of the superior vena cava are based on disorders of the left-right determination of the paired primitive veins in early embryonic development. The malrotation and malfixation of the bowel seem to be induced by the abnormal process of the embryonic duodenal loop with subsequent malpositioning of the portal vein and the superior mesenteric vessels. We demonstrate the wide variety of cardiac and extracardiac congenital malformations with considerable overlap in left and right isomerism. As a consequence, the differentiation between left or right isomerism may be sometimes difficult. Highly skilled sonographers and radiologists should be familiar with the corresponding embryonic development, which is essential for understanding the complex malformation pattern. Complete evaluation of each individual case is mandatory for adequate parental counselling with regard to further diagnostic steps and the planning of perinatal management.

P19.09: The syndrome of right atrial isomerism: prenatal diagnosis and outcome – 2 case reports with literature analysis

To describe the cardiac anomalies and outcome in the fetus with right atrial isomerism (RAI). The confirmed ultrasound result of fetuses with RAI: out of 88 cases, 2 examined in our hospital and the other 86 cases obtained from literature were analysed. Gestational age at diagnosis, the nuchal translucency, the karyotype were noted; Once the laterality, visceroatrial arrangement and cardiac position of the fetus have been established, the next step is to analyse the heart in a sequential segmental approach. The cardiac findings and outcome were noted. All these data were collected for analysis. Among 88 fetuses, 70 had a correct prenatal diagnosis of right isomerism. 80 showed different types of viscerocardiac heterotaxy, 79 of them in combination with juxtaposition of vena cava inferior and aorta. 85 had cardiac defects, with a high prevalence of atrioventricular septal defect (CAVSD) (n =68, 77%), right outflow tract obstruction (n =56, 64%), anomalous pulmonary venous return (n =49, 56%) and double outlet right ventricle (n = 23, 26%). 40 out of 49 fetuses with totally, or partially anomalous pulmonary venous drainage were overlooked by prenatal ultrasound examination. Only 26 children survived. The highest loss occurred in the neonatal period. Cardiovascular surgery was performed in 69% patients (n = 18), eight patients were inoperable. Biventricular repair was not possible in any of the patients. Supporting information for P01.05, P03.01, P03.05, P03.07, P03.10, P04.02, P04.03, P04.05, P04.07, P04.09, P05.05, P07.02, P07.03, P07.04, P08.09, P10.01, P10.02, P10.03, P10.04, P10.05, P10.06, P10.07, P12.01, P12.03, P12.04, P12.07, P14.04, P14.05, P14.09, P14.11, P17.03, P17.05, P17.07, P17.08, P17.09, P17.10, P17.11, P19.03, P19.06, P19.08

Segmental analysis of congenital heart disease: putting the "puzzle" together with computed tomography.

Advances in surgical and medical treatment for congenital heart disease have resulted in greater life expectancy. As a result, there has been an increase in the utilization of cross-sectional imaging for diagnosis and management of complex congenital heart disease. This manuscript describes a morphological and sequential segmental approach to deciphering the code of complex congenital heart defects in cross-sectional imaging, mostly computed tomography. This manuscript will review approaches to differentiate types of transposition, the anatomic relationships of cardiac structures, and the application of these relationships in the description of complex congenital heart disease.

Prenatal Ultrasound Screening of Congenital Heart Disease in the General Population

Congenital heart diseases (CHDs) carry a high prevalence rate in the general population (0.8%-1%). Most fetal CHDs occur in patients without any risk factors. The prenatal recognition of CHD has major impacts on the pregnancy and its outcome. The aforementioned data justify prenatal ultrasound (US) screening of CHD in the general low-risk population. As demonstrated in the literature, the application of an extended basic US cardiac examination improves the detection of CHD, in particular the conotruncal anomalies. The stepwise method suggested for fetal heart US screening during the mid-second trimester sonogram is based on 4 routine axial views of heart and great vessels: (1) a transverse view of the superior abdomen, (2) a 4-chamber view, (3) a 3-vessel view, and (4) a transverse view of the aortic arch. This protocol can be obtained rapidly because these scans are easy to perform. Despite the fact that the sequential segmental approach universally used in the postnatal diagnosis of CHD is not specifically addressed here, the detected anomalies can be categorized according to these views, and a short differential diagnosis proposed. Abnormal cardiac and/or vascular landmarks shown on these key scans should lead to a referral in the fetal cardiac center for a more precise evaluation, as well as for counseling.

Clinical value of fetal echocardiography at early and mid trimester of pregnancy in diagnosis for congenital

Fetal congenital heart disease can be identified reliably by prenatal echocardiography.A sequential segmental approach is critical for correct evaluation of the cardiac malformation.The outcome of the patients with congenital heart disease is poor and a multidisciplinary is needed to the parental counseling and perinatal management planning.It is of great significance for reducing rate of birth defect. Key words: Echocardiography; Fetus; Congenital heart disease

Pre- and Postoperative Evaluation of Congenital Heart Disease in Children and Adults with 64-Section CT

Although echocardiography is the imaging method of choice for diagnostic, preoperative, and postoperative evaluation of congenital heart disease, computed tomography (CT) is a helpful complementary imaging modality, particularly for postoperative evaluation. A thorough understanding of the normal anatomy and the morphologic features of congenital heart diseases is a prerequisite for choosing the optimal CT technique and achieving an accurate diagnosis. Furthermore, a close collaboration with a cardiologist with special training and expertise in congenital heart diseases is required. A sequential segmental approach should be used in evaluating morphologic features, especially during the review of CT images obtained in patients with rare congenital cardiac defects and in postoperative adult patients. To accurately document and interpret the altered flow conditions in patients with congenital heart disease, knowledge of the wide spectrum of surgical procedures and familiarity with the dedicated protocols for performing 64-section CT are needed.

Fetal echocardiography in diagnosing congenital heart disease prenatally: a multicenter clinical study

To evaluate the detection and accuracy of fetal echocardiography for congenital heart defects among high-risk populations. A prospective observational study of prenatal diagnosis of congenital heart disease was conducted in two tertiary obstetrics and gynecology hospitals between January 2003 and December 2004. Consecutive fetuses at risk of congenital heart disease underwent detailed fetal echocardiography during the study period. B-mode and colour/pulsed Doppler flow imaging were used in all cases. Follow-up was sought for all pregnancies. Indications for referral, maternal and gestational age at diagnosis, as well as prenatal and postnatal diagnosis were recorded prospectively. By comparing prenatal and postnatal diagnoses, sensitivity, specificity, and predictive values were estimated. A series of 2063 high-risk fetuses underwent detailed fetal echocardiography during the study period. The mean gestational age at examination was 26.5 weeks, ranging from 16 to 42 weeks. The most common indications for fetal echocardiography were advanced maternal age (31.7%), fetal arrhythmias (13.5%) and maternal infections (10.4%). Forty-three cases of fetal congenital heart disease were detected. The mean gestational age at prenatal diagnosis was 27.3 weeks ranging from 16 to 40 weeks. There were 3 false-negatives and 1 false-positive. The sensitivity, specificity, positive and negative predictive values were 92.1%, 99.9%, 97.2%, and 99.8%, respectively. Diagnostic accuracy was 86.1%. A cardiac defect suspected on routine prenatal sonography accounted for the highest proportion of abnormal cases (67.4%). As for pregnancy outcome, there were 24 (52.1%) terminations; 2.2% died in utero, 13% postnatally, and 28.3% survived. (1) Fetal congenital heart disease can be identified reliably by prenatal echocardiography. (2) Possible congenital heart disease or suspected heart defect noted on a screening obstetric sonogram is an important indication for fetal echocardiography. (3) A sequential segmental approach is critical for correct evaluation of the cardiac malformation. (4) The outcome of the patients with congenital heart disease is poor and a multidisciplinary approach is needed to the parental counseling and perinatal management planning.

Methodological study on real-time three-dimensional echo cardiography and its application in the diagnosis of complex congenital heart disease

Real-time three-dimensional echocardiography (RT-3DE) has made revolutionized improvements of cardiac imaging during the past few years. However, there is no standard examination method for RT-3DE so far. This study aimed to establish the diagnostic method of RT-3DE and evaluate its application in the diagnosis of complex congenital heart diseases (CHD). Fifty patients with complex CHD were examined by RT-3DE with modes of Live 3DE and Full Volume. A series of novel volumetric views combined with Van Praagh sequential segmental approach were introduced to reveal the pathological morphology of the hearts, which were compared with the findings of two-dimensional echocardiography (2DE), angiography and cardiac surgery. In 50 patients, 190 image acquisitions of Full Volume were performed at several acoustic windows including subcostal, apical and parasternal regions. Among them, 94.2% (179/190) of image acquisitions were successful. Most sectional volumetric views could be clearly displayed in 92.6% of the successful image acquisitions. However, sectional volumetric views could not be clearly displayed in 7.4%, which was mainly due to poor perspective conditions of examination location, improper instrument multi-parameter setting and insufficient information of whole heart captured in Full Volume acquisitions. As compared with surgical findings and angiography, RT-3DE made correction to the diagnoses in 2 cases including 1 with corrected transposition of the great arteries and the other with single atrium and mitral cleft. The diagnoses initially made by 2DE for these 2 patients were double outlet right ventricle with transposition of the great arteries and complete atrio-ventricular septal defect. RT-3DE can clearly display the pathological morphology of complex CHD by a series of novel volumetric views combined with sequential segmental approach through providing more spatial informative cardiovascular structures, which provides a practical method for RT-3DE diagnosis.

Simple approach to prenatal diagnosis of transposition of the great arteries

To analyze the most relevant anomalies, seen in a sequential segmental transverse views approach to imaging the fetal heart, that provide clues to the diagnosis of complete transposition of the great arteries (TGA). We reviewed retrospectively all the cases of isolated TGA diagnosed in our center or submitted for a second opinion through the spatio-temporal image correlation (STIC) telemedicine (TELE-STIC) program. Only transverse cardiac sweeps were obtained. Digital video clips and STIC volumes were reviewed. The abnormal features on four-chamber, five-chamber, three-vessel (3V) and three vessels and trachea (3VT) views were analyzed. The study population consisted of eight fetuses with TGA with normal extracardiac anatomy. The gestational age ranged from 13 to 32 (mean, 23) weeks. The maternal age ranged from 25 to 42 (mean, 32) years. A normal four-chamber view was seen in seven cases. Only one case demonstrated a significant ventricular septal defect. At the level of the five-chamber view a straight course arterial vessel arose from the left ventricle with lateral branches in all fetuses. In the 3V view, the ascending aorta was seen reaching more anteriorly than was the pulmonary artery in six cases. At the level of the 3VT view, two vessels (transverse aortic arch and superior vena cava) rather than three were seen in all cases. Our proposed sequential segmental approach to imaging the fetal heart apparently allows, in five-chamber and 3VT views, clear and confident signs to be detected that aid diagnosis of TGA.

Paralysis after aortic surgery: In search of lost cord function

During the early 1980s, the rate of paralysis after aortic surgery was as high as 41% in patients for the most complex thoracoabdominal aortic operations. After comparing human and chacma baboon (papio ursinus) spinal cord vascular anatomy, an animal model was established to study the pathophysiology of aortic cross-clamping and the aetiology of the paralysis. Techniques, including motor evoked responses for monitoring spinal cord function, were developed that were tried in humans and later culminated in prospective and randomized studies. These established that the following were protective: combining cerebrospinal fluid with intrathecal papaverine; cooling systemically to moderate or profound hypothermia; minimizing intercostal ischaemia time; using a sequential segmental repair approach; re-attaching all patent and segmental intercostal arteries below T8 for descending thoracic aortic repair and from T6 to L2 for thoracoabdominal repairs; continuing cerebrospinal fluid drainage for at least two days and maintaining patients hypertensive after surgery. The net result has been that, in two of our recent series, the risk of permanent paralysis has been reduced to between 3.1% and 3.8%.

Sequential segmental approach to fetal congenital heart disease.

The sequential segmental approach is now universally used in the diagnosis of congenital heart disease. Its utilization during fetal sonography has not been well described. In this review, we show how the fetal heart can be approached in a sequential segmental manner by using six basic sonographic views. The transverse view of the fetal upper abdomen is obtained to determine the arrangement of the abdominal organs, which, in most cases, provides the important clues to the determination of the atrial arrangement. The four-chamber view is obtained to evaluate the atrioventricular junctions. The views of the left and right ventricular outflow tracts are obtained to evaluate the ventriculoarterial junctions. The three-vessel view and the aortic arch view are obtained for the evaluation of the arrangement and size of the great arteries, which provides the additional clues to the diagnosis of the abnormalities involving the ventriculoarterial junctions and the great arteries. The standard protocol to acquire these six basic views is also introduced.

The management of symptomatic neonates with suspected congenital heart disease using combined cross-sectional echocardiography and pulsed Doppler flow study as the definitive investigations

We studied prospectively 140 consecutive symptomatic neonates with suspected congenital heart disease by combined cross-sectional and pulsed Doppler echocardiography. Using the sequential segmental approach, the anatomy at all cardiovascular junctions was clearly defined in 89 (64%) babies. Based on the non-invasive investigations, 47 went to surgery while 42 babies were treated medically. The other 51 babies underwent further cardiac catheterisation. Of these, only 23 (16%) required diagnostic catheterisation prior to their management decision. The remaining 28 babies were catheterised for (1) angiographic measurement of anatomical structures ( n = 12), (2) haemodynamic measurement ( n = 1), and (3) balloon atrial septostomy ( n = 15). One hundred and two babies had ultimate verification of their echocardiographic diagnoses. A total of 612 cardiovascular segments were identified. There were 33 (5%) echocardiographic errors, 23 of missed or uncertain diagnosis and 10 wrong interpretations. The diagnostic sensitivity and specificity for the combined non-invasive technique were thus 96 and 98%, respectively. Only one death was attributed directly to an echocardiographic error. Hence when the clinical outcome was taken into consideration, 88 of the 89 neonates without an initial catheterisation were judged to be appropriately managed for their presentation.

Classification of hearts with overriding aortic and pulmonary valves

Despite the clarity of the sequential segmential segmental approach to complex congenital heart malformations, the classification of hearts with overriding arterial valves remains contentious. A series of 67 hearts, all with overriding arterial valves, has therefore been studied in an attempt to provide clear and unambiguous criteria for their classification. There were 51 hearts with an overriding aortic valve, 13 hearts with an overriding pulmonary valve and 3 specimens with overriding of both valves. In each of these categories the degree of override and the underlying morphology varied considerably. The options to classify these hearts are limited. Using the “50 per cent rule” as a device to catalogue the type of connexion — irrespective of the morphology — all hearts were described in unambiguous fashion. A comparison with synonyms, as frequently used for purposes of classification, revealed that the latter are often insufficient properly to classify the basic abnormality. By classifying the type of connexion and describing the morphology separately, no basic problem remains in distinguishing between hearts with double outlet right ventricle and subpulmonary ventricular septal defect and hearts with the morphology of Fallot's tetralogy with an aorta almost exclusively arising from the right ventricle. Similarly, the classification of hearts with complete transposition and subpulmonary defect in the setting of the so-called Taussig-Bing heart is brought back to its proper perspective. Proper and consistent application of the sequential segmental approach leaves no room for ambiguity, even in complicated hearts with overriding arterial valves.

Double-inlet ventricle: Morphologic analysis and surgical implications in 32 cases

We analyzed, using a sequential segmental approach, 32 cases of double-inlet ventricle to assess the feasibility of surgical "correction" by either ventricular septation or a modified Fontan procedure. Twenty-two hearts had two atrioventricular valves, connected to a left ventricle in 19, a right ventricle in two, and a solitary indeterminate ventricle in one. Septation was possible in only 13. In contrast, the Fontan procedure seemed feasible in 20. The remaining 10 specimens had double inlet via a common valve to the left ventricle in two, the right ventricle in six, and an indeterminate ventricle in two. Seven of these had right atrial isomerism. Ventricular septation was not considered a possibility in these hearts. The Fontan procedure combined with atrial septation was a possibility in seven cases. From the morphologic stance, although the modified Fontan procedure seemed suitable in most cases, a significant number of hearts with two atrioventricular valves were suitable for ventricular septation.

Congenital Heart Diseases of the Cat

Feline congenital heart disease is less commonly encountered in veterinary medicine than acquired feline heart diseases such as cardiomyopathy. Understanding the wide spectrum of congenital cardiovascular disease demands a familiarity with a variety of lesions, occurring both in isolation and in combination, along with an appreciation of complex nomenclature and variable classification schemes. This review begins with an overview of congenital heart disease in the cat, including proposed etiologies and prevalence, examination approaches, and principles of therapy. Specific congenital defects are presented and organized by a sequential segmental classification with respect to their morphologic lesions. Highlights of diagnosis, treatment options, and prognosis are offered. It is hoped that this review will provide a framework for approaching congenital heart disease in the cat, and more broadly in other animal species based on the sequential segmental approach, which represents an adaptation of the common methodology used in children and adults with congenital heart disease.